Operational Policy for the Congenital Heart Disease Network
Royal Brompton Hospital Congenital Heart Disease Network
Authors: Dr Nitha Naqvi Dr Leonie Wong Dr Jan Till Dr Vicky Jowett Prof Julene Carvalho Dr Kostas Dimopoulos Lawrence Mack Michele Puckey Elizabeth Biggart Virginia Castro Meira Lynda Shaughnessy Penny Agent Frank Butau Maxine Ovens Simon Boote Approved by: Congenital Heart Disease Multidisciplinary Team Ratification Committee: Congenital Heart Disease Network Board
Date Ratified: 29/04/2019 Chairman: Dr Angela Tillett Implemented by: All Policy Authors Meeting Chair Meeting Members RBH-CHD Network MDT Network Management Team Issue Date: April 2019 Version: 001 Review Date: March 2022 Review interval: 3 years
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1 Contents
1 Contents...... 2 2 Introduction: The Royal Brompton Hospital ...... 7 3 Purpose: The RBH-CHD Network Operational Policy ...... 7 4 RBH Congenital Heart Disease Teams ...... 7 4.1 Network Management Team ...... 7 4.2 The Multidisciplinary Team ...... 8 4.3 Supporting Teams ...... 11 4.3.1 Paediatric Services ...... 11 4.3.2 Adult Services ...... 11 4.3.3 Theatres and Cardiac Catheter Laboratories ...... 11 5 The Operational Delivery Network ...... 11 5.1 Level 1 Centre (Network Host): ...... 11 5.2 Level 3 Centres & Specialist Paediatricians: ...... 12 5.3 Summary Outreach Activity ...... 13 5.4 Fetal / Perinatal Schedule ...... 14 5.5 Transplant Providers ...... 15 5.6 Pulmonary Hypertension Providers ...... 15 5.7 Academic Affiliation ...... 15 6 On-Call Support ...... 15 6.1 Paediatric CHD Contacts ...... 16 6.2 Adult CHD Contacts ...... 16 7 Level 1 Infrastructure & Services ...... 16 7.1 Outpatient Facilities ...... 16 7.2 Inpatient Beds ...... 16 7.3 ECMO ...... 17 7.4 Theatres ...... 17 7.5 Cardiac Catheter Laboratories & Invasive Diagnostics ...... 18 7.6 Non-Invasive Diagnostics ...... 19 7.7 Fetal Cardiology Service ...... 19 7.8 Electrophysiology Service ...... 20 7.9 Inherited Cardiac Consitions ...... 20 7.10 Paediatric Transplant Services ...... 20 7.11 Adult Transplant Services & Transition ...... 20 7.12 Adult Pulmonary Hypertension Service ...... 21
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7.13 Pregnancy & Heart Disease Centre ...... 21 7.14 Echocardiography Services ...... 22 7.15 Pharmacy ...... 22 7.16 Palliative Care ...... 23 7.17 Service Level Agreements: Non-Cardiothoracic Services ...... 23 7.18 Patient & Family: Access & Facilities ...... 24 7.18.1 Parking for patients and visitors ...... 24 7.18.2 Parking for disabled badge holders ...... 24 7.18.3 Reserved parking for outpatients ...... 24 7.18.4 Sydney Street Parking ...... 25 7.18.5 Congestion charge ...... 25 7.18.6 Public Transport ...... 25 7.19 Visitor accommodation at Royal Brompton Hospital ...... 26 7.19.1 Reservations ...... 26 7.19.2 Who Can Stay ...... 26 7.19.3 Accommodation Facilities ...... 26 7.19.4 Reception Opening Hours ...... 26 7.19.5 Free Wi-fi Service ...... 26 8 Patient Movement in the Network ...... 26 8.1 Clinical Management ...... 27 8.2 Children’s Acute Transport Service (CATS) ...... 27 8.3 South Thames Retrieval Service (STRS) ...... 27 8.4 Transition ...... 27 8.5 Escalation to Level 1 ...... 28 8.5.1 Outpatient Referral ...... 28 8.5.2 Level 1 Admission ...... 29 8.6 Seven Day Services Standards ...... 29 8.6.1 Self-Assessment ...... 30 8.6.2 RBH Board Assurance ...... 30 9 Quality and Governance ...... 30 9.1 MDT Meetings ...... 31 9.1.1 Adult CHD & JCC MDT Meetings - Timing and Venue ...... 31 9.1.2 Adult CHD & JCC MDT Membership & Quoracy ...... 31 9.1.3 Discussion Outcome ...... 33 9.2 Paediatric Ward Rounds ...... 34
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9.2.1 PICU / PHDU Ward Rounds ...... 34 9.2.2 CARE ...... 34 9.3 Adult Ward Rounds ...... 34 9.3.1 AICU / HDU Ward Rounds ...... 34 9.4 Image Sharing ...... 35 9.4.1 Image Exchange Portal ...... 35 9.4.2 Other Channels for Image Exchange ...... 35 9.5 Board and Subcommittee Meetings ...... 35 9.6 Approval Process: Network Documentation & Patient Information ...... 36 9.6.1 Development and Ratification ...... 36 10 Research ...... 37 11 Audit ...... 38 12 Patient Experience ...... 38 12.1 Patient Advice & Liaison Service (PALS) ...... 38 12.2 Support for In and Out Patients ...... 39 12.2.1 Safeguarding and Learning Disabilities Care ...... 39 12.2.2 Parents with Learning Difficulties ...... 40 12.2.3 Adult Clinical Psychology ...... 41 12.2.4 Paediatric Clinical Psychology ...... 41 12.2.5 Paediatric Play & Therapy ...... 42 12.2.6 Family Liaison ...... 42 12.2.7 Breastfeeding & Expressing Support Team (BEST) ...... 43 12.2.8 Chelsea Community Hospital School ...... 43 12.2.9 Bereavement Services ...... 43 12.2.10 Patient Information ...... 44 12.2.11 Brompton Fountain ...... 44 12.3 Contact Information ...... 44 12.3.1 Fetal CHD Contact and 24/7 Support ...... 45 12.3.2 Paediatric CHD Contact and 24/7 Support ...... 45 12.3.3 Adult CHD Contact and 24/7 Support ...... 45 12.3.4 Inherited Cardiac Conditions ...... 46 12.3.5 Palliative Care ...... 46 12.4 Feedback ...... 47 12.4.1 Friends and Family ...... 47
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13 Network Audit and Review ...... 48 13.1 Annual Audit Against QIs ...... 48 13.2 Annual Report ...... 49 13.3 Business Plan ...... 49 14 Appendix 1 – Transition Pathway ...... 50 15 Appendix 2 – Clinical Recommendations for Paediatric Cardiology ...... 51 15.1 Introduction ...... 51 15.2 Nomenclature ...... 51 15.2.1 Basic Investigations ...... 51 15.2.2 Other Investigations ...... 51 15.3 Timing of First Follow-Up Visit...... 51 15.4 Anomalous Left Coronary Artery from the Pulmonary Artery ...... 52 15.5 Anomalous Pulmonary Venous Drainage (total)...... 52 15.6 Aortic Valve Stenosis ...... 53 15.7 Aorto-Pulmonary Window ...... 54 15.8 Atrioventricular Septal Defect ...... 54 15.9 Coarctation of the Aorta ...... 55 15.10 Coronary artery fistulas ...... 56 15.11 Corrected Transposition (A-V and V-A discordance) ...... 57 15.12 Double Outlet Right Ventricle ...... 58 15.13 Fontan (Total cavopulmonary Connection (TCPC) pathway for complex lesions ...... 59 15.14 Hemitruncus arteriosus ...... 60 15.15 Hypertrophic Cardiomyopathy ...... 61 15.16 Hypoplastic Left Heart Syndrome ...... 61 15.17 Interrupted Aortic Arch ...... 62 15.18 Kawasaki Disease ...... 62 15.19 Patent Arterial Duct ...... 64 15.20 Pulmonary atresia and ventricular septal defect ...... 65 15.21 Pulmonary Valve Stenosis ...... 66 15.22 Pulmonary Atresia with Intact Ventricular Septum ...... 67 15.23 Secundum Atrial Septal Defect ...... 67 15.24 Fibromuscular Subaortic Stenosis ...... 68 15.25 Tetralogy of Fallot ...... 69 15.26 Complete transposition of the great arteries (intact ventricular septum) ...... 70 15.27 Truncus Arteriosus ...... 70
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15.28 Ventricular Septal Defect ...... 71 15.29 Authors ...... 72 15.30 References ...... 72 16 Appendix 3 – Management Guidelines for Adults with Congenital Heart Disease ...... 73 16.1 Physiological States ...... 73 16.2 Atrial Septal Defect ...... 74 16.3 Ventricular Septal Defect ...... 75 16.4 Atrioventricular Septal Defect ...... 76 16.5 Patent Ductus Arteriosus ...... 77 16.6 Congenital Valvular Aortic Stenosis ...... 78 16.7 Subaortic Stenosis ...... 79 16.8 Supravalvular Aortic Stenosis ...... 80 16.9 Coarctation of the Aorta ...... 81 16.10 Isolated Pulmonary Regurgitation after repair of Pulmonary Stenosis ...... 82 16.11 Right Ventricular Outflow Tract Obstruction ...... 83 16.12 Branch and Peripheral Pulmonary Stenosis ...... 84 16.13 Double-Chambered Right Ventricle ...... 85 16.14 Right Ventricle to Pulmonary Artery Conduit ...... 86 16.15 Congenital Mitral Stenosis ...... 87 16.16 Cor Triatriatum ...... 88 16.17 (Partial) Anomalous Pulmonary Venous Drainage ...... 89 16.18 Tetralogy of Fallot ...... 90 16.19 Ebstein Anomaly ...... 91 16.20 Truncus arteriosus ...... 92 16.21 Double Outlet Right Ventricle ...... 93 16.22 Fontan ...... 94 16.23 Transposition of the Great Arteries – Atrial Switch ...... 96 16.24 Transposition of the Great Arteries – Arterial Switch ...... 97 16.25 Congenitally Corrected Transposition of the Great Arteries ...... 98 16.26 Anomalous Aortic Origin of Coronary Artery ...... 99 16.27 PH and Eisenmenger Syndrome ...... 100 16.28 Reference ...... 100
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2 Introduction: The Royal Brompton Hospital
The Royal Brompton & Harefield NHS Foundation Trust (RBH) is the largest specialist heart and lung centre in the United Kingdom, and amongst the largest in Europe. The trust is formed of 2 sites, Royal Brompton Hospital and Harefield Hospital, following a merger in 1998. The cross-site partnership allows us to carry out some of the most complicated surgery and offer some of the most sophisticated treatment that is available anywhere in the world. Fetal, Paediatric and Adult Congenital Heart Disease (CHD) services are long established at RBH with a loyal Network of Level 3 centre doctors and allied professionals working together since the 1970s. Teaching and dialogue have always been recognised and RBH has a long history in educational courses in CHD attracting our own Network in addition to other professionals involved in CHD. The first “network days” were held in 2014. The CHD Network was formally recognised more recently in 2016. RBH appointed a CHD Network Director and an Associate CHD Network Director in 2018, followed by a CHD Network Manager in 2019.
3 Purpose: The RBH-CHD Network Operational Policy
This document will describe the RBH-CHD Network’s clinical and non-clinical team membership, how the team functions and how care is delivered across the patient pathway. It will outline structure, and policies and procedures that govern standardised, safe, sustainable and high- quality care. And it will outline agreed guidelines and treatment protocols throughout the network.
4 RBH Congenital Heart Disease Teams
4.1 Network Management Team
The network management team are responsible for all aspects operational delivery within the RBH-CHD Network. The current structure, and reporting lines, is illustrated below.
CHD Management Team Dr Nitha April 2019 Naqvi Network Director
Simon Dr Leonie Boote Wong Network Manager Assistant Network Director
Susana De Vacant Sousa Network Lead Nurse Network Admin
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4.2 The Multidisciplinary Team
The RBH-CHD Multidisciplinary Team (MDT) work together to provide high quality, comprehensive care to our CHD patients. The core MDT is listed in the table below. Where services are not co-located, service level agreements (SLAs) with neighbouring trusts ensure all aspects of care are met. A full list of Trust SLAs with neighbouring providers can be found in the section 7.8.
Speciality Surname Given Name Adult Paed WTE Comments
Hoschtitzky Andreas 1.0 Kassai Imre 1.0 Replacement TBC
Michielon Guido 1.0
Cardiac Cardiac Surgery Shore Darryl 1.0
Bautista Carlos 1.0
al Director of Paediatric Fraisse Alain Emile 1.0 Cardiology, Intervention Lead Kempny Aleksander 1.0 PHT Rigby Michael Lawrence 1.0
Consultants Intervention Slavik Zdenek 1.0 Malik Iqbal 1.0 (Imperial Healthcare)
Ernst Sabine 1.0
Wong Tom 1.0 Lead for Electrophysiology Clague Jonathon 1.0 Till Jan 1.0 Paediatric Co-Director
Consultants
Roses-Noguer Ferran 1 clinic / month
EP CHD Assistant Network Wong Leonie 1.0 Director
Bartsota Margarita 1.0
Carvalho Julene Souza 1.0 Lead for Fetal
Jowett Victoria 1.0 Fetal Consultants Tsai-Goodman Beverly 1.0 Babu-Narayan Sonya 1.0 MRI Imaging Daubeney Piers Edward Francis 1.0
Dimopoulos Konstantinos 1.0 Transition Lead Di Salvo Giovanni 1.0 Lead for Paediatric Research Fachini Spada Maraisa 1.0
Franklin Rodney Cyril George 1.0 Consultants Furck Anke 0.2 0.8 in PICU Gatzoulis Michael Athanassios 1.0 Adult CHD Lead Cardiac Krupickova Sylvia 1.0 MRI Imaging Li Wei 1.0 Echocardiology Naqvi Nitha 1.0 CHD Network Director
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Nienaber Christoph Anton Theodor 1.0 Pantazis Antonios 1.0 Prasad Sanjay 1.0 Rowlinson Giselle Victoria 1.0 Rafiq Isma 1.0 Pires Ana 1.0 Alam Haraith 1.0 Mohiaddin Raad 1.0 MRI Lead for CHD Rybicka Justina 1.0 HF & Transplant
Wort John 1.0 PHT Lead
McCabe Colm 1.0
PHT ants
Consult Price Laura 1.0
Burmester Margarita 1.0 Director PICU
Macrae Duncan 1.0 Shastri Nitin 1.0 Gala-Peralta Sandra 1.0 Furck Anke 0.8 0.2 in Cardiology Casanueva Lidia 1.0 Armaburo Angela 1.0 Narayanan Abhishek 1.0
Desai Ajay 1.0 PICU Consultant Intensivists Consultant PICU Chan-Dominy Amy 1.0
Burmester Stephane 1.0
Price Susanna 1.0 Doyle James 1.0 Morgan Cliff 1.0 Passariello Maurizio 1.0 Patel Brijesh 1.0 Singh Suveer 1.0 Thakuria Louit 1.0
ICU Consultant Intensivists Consultant ICU Weaver Christine 1.0 A Xu Tina 1.0
Jaggar Sian 1.0
Aw Tc 1.0 Lane Mary 1.0 Mele Sara 1.0 Keogh Brian 1.0 O’Donoghue Cathy 1.0 Bianchi Paulo 1.0
Pickworth Tom 1.0 Consultant Anaesthetists Consultant Ghori Arshad 1.0
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Semple Thomas 1.0 CT Rubens Michael 1.0 CT
Baltabaeva Aigul 1.0 PH Imaging Imaging
Consultant Deveraj Anand 1.0 PH
Harcourt Jonathan 1.0 ENT, Imperial Healthcare
Al Yaghchi Chad 0.2 ENT, Imperial Healthcare
Homfray Tessa Frances Rose 0.6 Genetics Other
Dijk Fleur 0.15 Genetics (Hon Cont.) Consultants Ghali Neeti 0.15 Genetics (Hon Cont.)
De Sousa Susana 1.0 Lumanisakio Julietta 1.0
McCarthy-Morogh Sarah 1.0
Fetal CNS CNS Fetal Education
Team & Fetal Fetal & Team Moura Pilar 1.0 Cox Fran 1.0 Meryon Rosanna 1.0
Karagadova Elena 1.0
Team & & Team Education Paediatric Paediatric Damant Francesca 1.0 Paediatric CNS CNS Paediatric Johnson Liz 0.8 Shaughnessy Lynda 1.0 SN, ACHD Lead Finlay Clare 1.0 McDonnell Ella 0.75
Education Rusike Fortunate 1.0 Team & ACHD ACHD & Team Adult CHD CNS CNS CHD Adult Warsama Suad 0.5
Harries Carl 1.0 Lead CNS
PH PH Barbosa Joana 1.0
PH CNS CNS PH Team & & Team
Education Craig Stuart 1.0
Castro Meira Virginia 1.0 Paediatric LN, CHD Lead Haidu Loredana 1.0
Fox Jos 1.0
Education
Transition Transition Transition CNS Team & & Team CNS Cottam Adrienne 1.0 Renwick Catherine 1.0 LN, ICC & Electrophysiology Green Lucy 1.0 Collins Sami 1.0 Genetic Counsellor
Education Cowley Bethan 1.0
ICC CNS Team & Team CNS ICC MacKay Rachael 0.6
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4.3 Supporting Teams
RBH has a full range of services and specialities that support our CHD patients. This section will summarise staffing in key areas as identified by CHD Level 1 quality indicators provided by Quality Surveillance.
4.3.1 Paediatric Services
RBH has a 2.0 WTE Paediatric Clinical Psychologists who will see paediatric heart patients. However, the trust employs 5.0 WTE paediatric psychologists so there is a good degree of cross- cover. RBH has 2.0 WTE Data Collection Officers (shared with adults), 7.35 WTE Play Specialists, a named contact for bereavement in the Bereavement Team, and a named contact for safeguarding. Please refer to section 12 of this document for specific contact information.
4.3.2 Adult Services
RBH has a 1.0 WTE Consultant Clinical Psychologist whom will see adult cardiac patients. However, the trust employs 6.43 WTE adult psychologists so there is a good degree of cross- cover. RBH has a named contact for bereavement in the Bereavement Team, a named contact for palliative care in the palliative care team, and a named contact for safeguarding. Please refer to section 12 of this document for specific contact information.
4.3.3 Theatres and Cardiac Catheter Laboratories
The RBH Theatres Team consists of 39 WTE Nurses, 5 of which are recognised as Surgical Practitioners. The RBH Catheter Labs are served by 21 WTE Nurses. There are also shared nurses and health professionals from imaging, nuclear medicine and cardiac physiology in Theatres and the Labs. Across both departments there are 17 WTE Operating Department Practitioners and 14 WTE Perfusion Scientists (9 of whom are Band 8a or above). The lead for RBH Perfusion is Tim Jackson.
5 The Operational Delivery Network
The RBH-CHD Network is hosted by the Royal Brompton Hospital, Royal Brompton & Harefield NHS Foundation Trust. There are currently twenty-six Level 3 DGH’s in the network with a wide geographic spread across the South-East.
5.1 Level 1 Centre (Network Host):
Royal Brompton Hospital – Network Hub, Level 1 Paediatric and Adult Surgical Centre
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5.2 Level 3 Centres & Specialist Paediatricians:
The following table lists the hospitals in the RBH-CHD Network and the Paediatricians with Expertise in Cardiology (PEC) that deliver outreach clinics.
No. Hospital (H), Trust (T), PEC (P) No. Hospital (H), Trust (T), PEC (P) H: Basildon University Hospital H: Broomfield Hospital T: Basildon & Thurrock University Hospital NHS FT T: Mid-Essex Hospital NHS T 1 2 P: Dr Ramaratnan Ramanan P: Dr Mahesh Babu P: Dr Rani Thankappen P: Dr Job Cyriac H: Chelsea & Westminster Hospital H: Colchester Hospital T: Chelsea & Westminster Hospital NHS FT T: East Suffolk & North Essex NHS FT 3 4 P: Dr Angela Tillett P: Dr Bikash Bhojnagarwala P: Dr Ramona Onita H: Croydon University Hospital H: East Surrey Hospital (Redhill) T: Croydon Health Services NHS T T: Surrey and Sussex Healthcare NHS FT 5 6 P: Dr Arun Kumar P: Dr Majeed Jawad P: Dr Grant Marais P: Dr Prashanthi Katta H: Hammersmith Hospital H: Hemel Hempstead Hospital T: Imperial College Healthcare NHS T T: West Hertfordshire Hospitals NHS T 7 8 P: Dr Emmanuel Quist-Therson P: Dr Jayanta Banerjee P: Dr Sankara Narayanan H: Hillingdon Hospital H: Kingston Hospital 9 T: The Hillingdon Hospitals NHS FT 10 T: Kingston Hospital NHS FT P: Dr Abbas Khakoo H: Lister Hospital H: Maidstone Hospital T: East & North Hertfordshire NHS T T: Maidstone & Tunbridge Wells NHS FT 11 12 P: Dr Jonathan Kefas P: Dr Krishnan Balasubramanian P: Dr Anshoo Dhelaria H: Northwick Park Hospital H: Princess Alexandra Hospital T: London North West University Healthcare NHS T: The Princess Alexandra NHS FT 13 P: Dr Gerald Hanson 14 P: Dr Richard Nicholl P: Dr Sanjay Raina P: Dr Sathish Bangalore H: Queen Charlottes & Chelsea Hospital H: Queen Elizabeth II Hospital T: Imperial College Healthcare NHS T T: East & North Hertfordshire NHS T 15 16 P: Dr Jonathan Kefas P: Dr Jayanta Banerjee P: Dr Anshoo Dhelaria H: Queen Mary's Hospital for Children H: Queen Mary's University Hospital T: Epsom & St Helier University Hospital T: St George's University Hospital NHS FT 17 18 P: Dr Anay Kulkarni P: Dr Ruth Shephard P: Dr Donovan Duffy
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H: Southend University Hospital H: St George’s Hospital T: Southend University Hospital NHS FT T: St George's University Hospital NHS FT 19 P: Dr Mokhlesur Rahman 20 P: Dr Anay Kulkarni
P: Dr Donovan Duffy P: Dr Vineet Gupta H: St Mary's Hospital H: St Peter's Hospital T: Imperial College Healthcare NHS T T: Ashford & St Peter's NHS FT 21 22 P: Dr Alison Groves P: Dr David Inwald P: Dr Tosin Otunla H: Watford General Hospital H: West Middlesex University Hospital T: West Hertfordshire Hospitals NHS T T: Chelsea & Westminster Hospital NHS FT 23 P: Dr Emmanuel Quist-Therson 24 P: Dr Eleanor Hulse P: Dr Sankara Narayanan H: West Suffolk Hospital H: Worthing Hospital T: West Suffolk NHS FT T: Western Sussex Hospitals NHS FT 25 26 P: Dr Martina Noone P: Dr Anil Garg
5.3 Summary Outreach Activity
There are a large number of outreach clinics per year across all the hospitals listed in the Level 3 table (5.2). Apart from Kingston, every hospital has established outreach and at least 1 PEC. The table below shows which RBH Consultants support each centre, and how often joint clinics are held per year.
Consultant Outreach Clinic Visits / Year St Mary's Hospital (Imperial) 6 Prof Fraisse Princess Alexandra Hospital 5 St Peter's Hospital 4 Chelsea & Westminster Hospital 10 Prof Daubeney Maidstone Hospital 7 Queen Mary's Hospital for Children 5 Basildon University Hospital 6 Basildon University Hospital (Telemedicine) 12 Southend University Hospital 6 Southend University Hospital (Telemedicine) 12 Dr Rigby Colchester Hospital (with Dr Bartsota) 5 Colchester Hospital (Telemedicine) 5 Croydon University Hospital 5 Worthing Hospital 4
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East Surrey Hospital 12 Dr Naqvi West Suffolk Hospital 4 Dr Krupickova Chelsea & Westminster Hospital 26 Dr Krupikcova & Dr Spada Broomfield Hospital 5 Hillingdon Hospital 12 Dr Franklin Northwick Park Hospital 12 Queen Elizabeth II Hospital 6 Watford General Hospital 12 Dr Slavik Watford / West Mid at Harefield 12 West Middlesex University Hospital 12 Dr Slavik or Dr Furck Hemel Hempstead Hospital 12 Hammersmith Hospital 48 Harefield Hospital 48 Dr Jowett (Fetal) Queen Charlottes & Chelsea Hospital 60 Watford General Hospital 12 Northwick Park Hospital 12 Dr Till (with Dr Wong) St George's University Hospital 12 Colchester University Hospital 4 Dr Bartsota Queen Charlottes & Chelsea Hospital 42 Chelsea & Westminster Hospital 42 Dr Furck Queen Mary's University Hospital 12 Dr Spada Chelsea & Westminster Hospital 12 Dr DiSalvo Chelsea & Westminster Hospital 21 Prof Carvalho (Fetal) St George's University Hospital 80 Dr Tsai-Goodman (Fetal) St George's University Hospital 70
Every outreach clinic has clinical nurse specialist support.
5.4 Fetal / Perinatal Schedule
The table below show weekly scheduled fetal cardiology clinics at RBH and our three fetal medicine hubs in London; Chelsea and Westminster, Queen Charlotte’s and Chelsea, and St George’s hospitals.
Hospital Monday Tuesday Wednesday Thursday Friday Chelsea & Westminster X X Queen Charlotte's X X X X Royal Brompton X X X St. George's X X X X X
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5.5 Transplant Providers
Adult – Harefield Hospital Paediatric – Great Ormand Street Hospital for Children NHS Foundation Trust
5.6 Pulmonary Hypertension Providers
Adult – Royal Brompton Hospital Paediatric – Great Ormond Street Hospital for Children NHS Foundation Trust
5.7 Academic Affiliation
Imperial College Healthcare NHS Trust
6 On-Call Support
Many paediatricians with expertise in cardiology (PEC’s) and adult network consultants have the phone numbers of paediatric and adult consultant cardiologists at RBH. There is always a consultant cardiologist on-call, network consultants will usually contact them for direct advice. When a CHD patient needs urgent review, or advice is needed by a consultant in a network hospital, the on-call cardiology registrar will receive the request for advice or assistance. This service can be reached via our switchboard and asking for the ‘on-call paediatric cardiology registrar’, or the ‘on-call adult cardiology registrar’; both can be contacted by bleep. All registrars have an @NHS.net email account which can be provided. There is also dedicated fax number. The on-call Adult CHD cardiologist also provides a 24/7 service to our Cardiac Obstetric Service which is run jointly with Chelsea & Westminster Hospital. Referring obstetricians and PEC’s also have contact details for fetal cardiologists to discuss urgent cases and pre and perinatal management plans. Relevant consultant on-call rosters and associated frequency are as follows: - Paediatric Cardiology 1 in 7 - Adult CHD 1 in 5 - Adult & Paediatric Intervention 1 in 4 - CHD Surgery 1 in 4
During working hours there is an additional layer of support from our Fetal, Paediatric and Adult CHD Clinical Nurse Specialist Team. Contact details for our nurse specialist team can be found in Section 12.
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6.1 Paediatric CHD Contacts
Main Switchboard +44 (0)20 7352 8121 Registrar On-Call 07969 266194 / 1233 (Phone / Bleep) Rose Ward 82412 / 88543 / 82411 PICU (Amanda Jones) 82400 / 82401 / 82402 / 82411 PICU Fax +44 (0)20 7351 8503
6.2 Adult CHD Contacts
Main Switchboard +44 (0)20 7352 8121 Registrar On-Call 1188 (Bleep) Princess Alexandra Ward 82515 York Ward 82542 York Ward (HDU) 82540 / 82541 Paul Wood Ward 82500 / 82501 Elizabeth Ward (HDU) 82530 / 82531 / 82524 / 82525 AICU 82331 / 82332
7 Level 1 Infrastructure & Services
RBH service specification is such that it allows the delivery of the highest quality of care to our CHD Patients and their families. Section 7 will outline key structure and services that enable the hospital to exceed our patient’s expectations, reassure our network partners, and realise our RBH- CHD Strategic Vision (available on the RBH-CHD Webpage).
7.1 Outpatient Facilities
Royal Brompton Hospital outpatient department is in Fulham Wing / South Block. This building is separate from the main hospital building on Sydney Street, and is located nearby on Fulham Road. Most outpatient appointments will be in the Fulham Wing / South Block, regardless of specialism. There are approximately 115 fetal CHD clinics per year, 400 paediatric CHD clinics per year, and 400 adult CHD clinics per year. Most clinics will have 1 or more rooms attached to them so the service can flex up and down to meet demand.
7.2 Inpatient Beds
RBH has 304 inpatient beds in total, this is broken up into the following: - AICU 18 beds - Foulis Ward (Respiratory / CF) 35 beds - Elizabeth Ward (HDU) 26 beds - Lind Ward (Respiratory Day Case) 35 beds - PICU 20 beds (4 of which are HDU beds) - Paul Wood Ward 31 beds
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- Princess Alexandra 35 beds - Rose Ward (Paediatrics) 31 beds (4 of which are HDU beds) - Victoria Ward (Respiratory) 19 beds - York Ward 16 beds - Other / Diagnostic / Day Case 38 beds RBH-CHD Network patients are supported by our adult and paediatric intensive care units. The hospital has seven dedicated adult critical care consultants and one of the country's largest academic research programmes in intensive care medicine.
7.3 ECMO
Extracorporeal membrane oxygenation (ECMO) supports recovery after major life-saving surgery or life-threatening illness by oxygenating blood outside of the body and returning it into circulation. RBH has an established ECMO service for both adult and paediatric patients that includes full retrieval. ECMO in PICU is predominantly cardiac ECMO, neonatal and paediatric. Occasionally, RBH will initiate respiratory ECMO if other national centres are full. All PICU consultants are trained to manage ECMO patients, and we have 20 PICU ECMO Nurses. The service treats between 10 to 17 patients per year. The Adult ECMO service is 1 of only 5 NHS-commissioned ECMO Centres for severe acute respiratory failure in the country. Following a redesign in 2016 / 17, in conjunction with Guy’s & St Thomas’s NHS Foundation Trust, the service has increased capacity and greater multidisciplinary collaboration. The Adult ECMO service was granted the Platinum Award by the Extracorporeal Life Support Organisation (ELCO) 2019. The ECMO service across both adults and paediatrics is compliant with national educational and training standards and have established, robust submission to national audit. Referral to RBH ECMO Services can be made via referral form at https://www.rbht.nhs.uk/our- services/clinical_support, emailing the ECMO Team at [email protected], calling the hospital and asking for the ECMO Team (+44 (0)20 7351 8121), or calling the ECMO Nurse mobile on +44 (0)7815 494175. Advice about ECMO and referral criteria is available on the web page or over the phone. Other, non-urgent ECMO enquiries should be emailed to [email protected].
7.4 Theatres
The theatres' team provides a seven-day, 24-hour service with staff in full and part-time positions and with a strong on-call commitment. There are five dedicated cardiothoracic operating theatres and one hybrid theatre on the Brompton Site. Certain theatres are earmarked for paediatric use. Specialities covered include thoracic and cardiac for paediatric and adult patients, although we also perform other more general procedures such as dental, gynaecology and obstetrics, ear, nose and throat (ENT) or general surgical cases
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when patients' complex cardiac medical condition means they would be best managed by our specialist cardiology teams. Our theatres see over 460 surgical interventions in CHD alone. The staff within the department include:
- nurses - operating department assistants / practitioners - anaesthetic nurses - perfusionists - healthcare assistants - porters - surgical assistants - cardiac physiologists
Surgery performed includes:
- coronary artery bypass graft - robotic cardiac surgery - valve surgery - complex congenital heart surgery - minimally invasive thoracic surgery - paediatric and neonatal operations - major thoracic and oesophageal surgery.
In conjunction with Chelsea and Westminster Hospital, we also perform high-risk obstetrics (caesarean sections on our acquired congenital patient population).
7.5 Cardiac Catheter Laboratories & Invasive Diagnostics
The cardiac catheter labs provide adult and paediatric diagnostic and intervention services as well as treatment for arrhythmias using electrophysiological studies and devices. The department is composed of five cardiac catheter labs. Three of these labs are single-plane facilities while the remaining two are biplane facilities. One of the biplane labs is used for paediatrics and adult congenital heart patients. Two of the catheter labs are dedicated especially to electrophysiology. The lab sees over 630 electrophysiology and coronary interventions per year in CHD alone. Each case in the cardiac catheter lab is supported by a team that includes an operator, nurse, radiographer and cardiac physiologist. In some cases, an anaesthetic nurse and operating department assistant/practitioner from the theatre's department will also be included. The operator, either a cardiology consultant or specialist registrar (SpR), is responsible for placing the catheters, putting in stents and carrying out the study itself. The nurse sets up the trolleys, administers drugs and looks after the patient before and after the procedure. A scrub nurse ensures cleanliness and delivery of appropriate equipment throughout the procedure. The
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radiographer is trained in the use of ionized radiation. He or she is responsible for moving equipment, setting doses to keep radiation low, and ensuring radiation safety in the labs. The cardiac physiologist measures and records electrocardiograms (ECGs) and blood pressure during procedures, as well as building a report based on what catheters are used and when they are placed. RBH cases diagnosed and treated in the catheter labs include:
- Diagnostic Angiogram - Percutaneous Coronary Intervention (PCI) - Pacemaker (PPM) - Implantable cardioverter defibrillator (ICD) - Cardiac resynchronisation therapy pacemaker & defibrillator (CRT-P / CRT-D) - Electrophysiology and ablation - Magnetic navigation directed ablation - Paediatrics diagnostics and intervention - Adult diagnostics and intervention - Transfemoral aortic valves implantations - Mitraclip program for repairs
7.6 Non-Invasive Diagnostics
Non-invasive diagnostics at RBH are comprehensive. Our laboratories can offer clinical biochemistry, genetics and genomics, haematology, histopathology / cytology, microbiology and tissue typing. RBH imaging has always been an area of significant investment, the hospital offers plain x-rays (including mobile x-ray), barium studies, ultrasound scanning including complex echocardiography, nuclear scans and tests, CMR, Cardiac CT scanning and CT Coronary Angiography and stress testing. The hospital also has full service physiology departments offering lung physiology, cardiac physiology (including pacing, 24-hour tapes, event recorders and exercise testing), sleep diagnostics and a muscle laboratory.
7.7 Fetal Cardiology Service
The Brompton centre for fetal cardiology comprises The Royal Brompton and three linked tertiary fetal medicine units: St Georges Hospital, Queen Charlottes Hospital and Chelsea and Westminster Hospital. A comprehensive fetal cardiology service is provided for patients at increased risk of congenital heart disease and the provision of 14 clinics/week allows fast track appointments for suspected cardiac abnormalities detected at the 20-week obstetric anomaly scan. Integration with fetal medicine allows rapid comprehensive assessment of the fetus including extracardiac abnormalities, invasive testing and genetic counselling. The service is supported by 3 dedicated fetal clinical nurse specialists who provide ongoing contact and support for patients, parent education days and transition through to postnatal services.
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There is a well-developed system to allow rapid access to fetal cardiology appointments for patients with suspected congenital heart disease at screening. The process for referral into fetal cardiology is outlined in section 8.
7.8 Electrophysiology Service
The paediatric and fetal electrophysiology service operates from the RBH site but services the entire Network with good communication links. Children can be seen in the dedicated arrhythmia clinic lead by the paediatric electrophysiology consultants, but care is shared between Network Paediatricians with expertise (PECS) whenever possible. There are 2 paediatric electrophysiology consultants who run both the routine elective service with outpatient and inpatient care and provide emergency advice for the on-call paediatric cardiologist, fetal cardiologist or Network PECS. The service provides comprehensive care including; ECG interpretation, 24-hour tapes, event monitors and exercise tests, tilt tests, diagnostic electrophysiology and ablation, laser lead removal and cardiac sympathectomy. There are excellent vertical links and transition to the adult congenital electrophysiology service with joint MDT and catheter lab working.
7.9 Inherited Cardiac Consitions
The paediatric inherited cardiac conditions service is based at both RBH and Harefield sites. There are currently 5 consultants involved in running this comprehensive service for children suspected of having a cardiomyopathy, aortopathy or channelopathy. Consultant medical care is enhanced by a team of specialist nurses who guide and assist lifestyle and offer transition to adult services. The service offers both family and multidisciplinary clinics. The service is co-located with a genetic science lab and is complemented by a team of consultant geneticist, genetic counsellors, bioinformaticians and genetic scientists. Weekly MDT meetings to discuss and consolidate care are in place.
7.10 Paediatric Transplant Services
RBH is supported by the Great Ormond Street Hospital for Children NHS Foundation Trust Cardiothoracic Transplant Unit. The Great Ormond Street unit is the biggest of its kind in Europe for paediatric heart transplant with a dedicated team performing between 20 to 30 heart transplants per year. The service offers full perioperative assessment as well as follow-up through to adolescence. To complement RBH’s partnership with the Great Ormond Street, RBH has a dedicated Transplant Transition service based at Harefield Hospital.
7.11 Adult Transplant Services & Transition
The RBH heart and lung transplantation unit, based at Harefield Hospital, is the UK’s largest and most experienced centre for heart and lung transplants. The unit works with the Heart Science Centre, which is at the forefront of research into heart disease and transplantation.
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Harefield’s dedicated transplant unit consists of two wards, with a total of 34 beds, most of which are in single or double rooms. Patients are admitted for transplant assessment, routine check-ups and unplanned admissions in the event of post-transplant complications (such as infection or organ rejection). There is a dedicated transplant follow-up clinic and a focus on enabling patients to live the most normal and active life possible. The service also has a dedicated Transplant Transition Team who regularly accept teenagers and young adults into adult care. Transplant services at Harefield Hospital are supported by our specialist immunosuppression monitoring services, which includes the UK National Monitoring Service for Sirolimus.
7.12 Adult Pulmonary Hypertension Service
The pulmonary hypertension (PH) service at RBH is run by the Royal Brompton pulmonary vascular unit. It's one of seven centres that make up the UK’s national pulmonary hypertension service. The multidisciplinary team provides care for patients with pulmonary hypertension which includes:
- a full diagnostic investigation - inpatient and outpatient management of pulmonary hypertension long-term follow-up. RBH patients are mainly from the south of England and Wales, but as a tertiary centre for Adult CHD, RBH also sees patients from across the UK. The team have developed a shared care service with St George’s Hospital, Southampton General Hospital, The John Radcliffe Hospital in Oxford, Royal Berkshire Hospital, Reading, Milton Keynes Hospital, Surrey and Sussex Hospital (at Redhill), and St Richard’s hospital in Chichester.
7.13 Pregnancy & Heart Disease Centre
The aim of this service is to provide a gold standard care to enable women with pre-existing cardiac conditions, or a cardiac condition which arise during pregnancy, to achieve a successful pregnancy and birth. This is a specialist multi-disciplinary centre led by consultant cardiologists and obstetricians. The service is provided jointly with the high-risk maternity team from Chelsea & Westminster Hospital. There are 2 clinics offered as follows:
- Pre- conception Counselling Clinic Monday afternoon 2-5pm RBH
- High Risk Cardiac Obstetric Clinic Tuesday morning 9am-12.40pm Chelsea & Westminster Hospital
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Details of how to refer into this service are in Section 8
7.14 Echocardiography Services
The department of echocardiography provides a specialist service in the diagnosis and management of cardiovascular and thoracic disease. There are 17 WTE Adult Echocardiographers and 6 WTE Paediatric Echocardiographers. RBH is committed to deliver the best possible service and care to our patients. Investigations available at the department of echocardiography:
- Transthoracic echocardiography - Transoesophageal echocardiography - Adult congenital echocardiography - Contrast echocardiography - 3D transthoracic and transoesophageal echocardiography - Dobutamine stress echocardiography - Physiological (exercise) stress echocardiography - Dyssynchrony echocardiography - CRT optimisation - Paediatric echocardiography
Adult Echocardiography Lead Cathy West (EACVI)
Paediatric Echocardiography Lead Manjit Josen (EACVI)
7.15 Pharmacy
The clinical pharmacy service is provided by directorate teams consisting of a clinical specialist pharmacist, a junior pharmacist and in some directorates, a technician. Practice research and drug usage reviews are actively encouraged within the directorates and are led by our specialist pharmacy team. The department is actively involved in patient education rehabilitation programmes for patients following cardiac surgery and for those with heart failure as well as individual patient medication consultations. Technical services staff at RBH provide a central intravenous additive service to respiratory and paediatric patients as well as adult and paediatric TPN and clinical material for both sites, using a combination of external manufacturers and use of the aseptic unit at Royal Marsden Hospital.
The dispensary provides inpatient, outpatient and discharge medication services to the wards and departments. A chief technician and a senior pharmacist are responsible for the daily management of the dispensary. Two medicines information pharmacists, assisted by a principal technician and a rotational pharmacist, provide the Trust's medicine information service using online facilities and databases. The unit also runs a patient helpline service, the direct dial number is +44 (0)20 7351 8901. The
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medicines information unit is the UK recognised centre of excellence in cardiothoracic thoracic medicine information.
7.16 Palliative Care
RBH supportive and palliative care aims to improve the quality of life of patients and their families facing problems associated with advanced or life-threatening illness. This includes the management of symptoms, social and psychological concerns. RBH has a supportive and palliative care team consists of palliative care consultants, clinical nurse specialists and Macmillan nurses. The team provides an advisory service to patients throughout the Trust. They support patients to make decisions about their health and future care. They will assess individual needs and work with medical teams to improve symptoms and quality of life. The team will also help coordinate patient care with all the other health professionals involved. If required, the they can arrange referrals to other professionals within the hospital or in the community. The palliative care team’s key aims are to:
- offer specialist advice in managing breathlessness and other symptoms, including pain and nausea - inform about other services that might be helpful to a patient, such as community services and support groups - assist with any psychological, social and/or spiritual concerns - provide information and advice about every aspect of care and treatment, including financial and benefits advice - support family, friends or carers - give practical advice on medication management and written information on symptom management - help patients plan ahead to ensure their wishes are known
Contact information for our Palliative Care Team can be found in section 12.
7.17 Service Level Agreements: Non-Cardiothoracic Services
SLAs are in place for the provision of non-cardiothoracic clinical support to RBH from Chelsea & Westminster Hospitals NHS Foundation Trust and cover the following clinical specialties: Adult services: Paediatric services:
- Neurology - Safeguarding Children - Diabetes and Endocrinology - Endocrinology - General Surgery - Gastroenterology
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- Gynaecology - General Surgery - Obstetrics - Urology - Gastroenterology - Dental Surgery - Orthopaedics - Dermatology - Ophthalmology - Neonatology - Cardiology - Nephrology (Renal) - Psychiatry - Neurology - HIV phone service - Urology
An SLA is also in place for the provision of Ear, Nose and Throat services from Imperial College Healthcare NHS Foundation Trust.
7.18 Patient & Family: Access & Facilities
Full patient and visitor information about accessing RBH is available on the trust web site at https://www.rbht.nhs.uk/patients-visitors/patients. Hard copies of this information are available in outpatients, inpatient units and can be provided on request.
7.18.1 Parking for patients and visitors
RBH has excellent public transport links, details can be found later in this section. There is limited parking at RBH and limited pickup and drop off area at Sydney Street. There is metered, on-street parking but in central London, street parking is very limited and not guaranteed. There is a public car park three minutes' walk away on Sydney Street, near King's Road and managed by CP Plus Parking. RBH can offer free parking to anyone unable to use public transport. This can be arranged by calling +44 (0)20 7351 8012.
7.18.2 Parking for disabled badge holders
A small number of disabled parking spaces are available in:
- Dovehouse Street - Dudmaston Mews (these are controlled by the council and not bookable through the Trust) - Cale Street - Foulis Terrace - Sydney Street, in front of the main hospital entrance. These two bays are limited to four hours and cannot be reserved.
7.18.3 Reserved parking for outpatients
We have five parking spaces in Dudmaston Mews, behind Fulham wing/South Block, available to reserve (by calling +44 (0)20 7351 8012). This must be done within two weeks of your outpatient
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appointment and are subject to availability. These are separate to the disabled parking spaces in Dudmaston Mews controlled by the council. Parking spaces are only available to outpatients coming in for their appointments. Subject to availability, these five parking spaces in Dudmaston Mews can be used by visitors after 4 pm Monday to Friday. Use of the five bays is free of charge. Otherwise, Dudmaston Mews is a pay- and-display area on Saturdays and parking on Sundays is free.
7.18.4 Sydney Street Parking
There is no car parking available in the Sydney Street area of the hospital other than four disabled spaces, available on a first-come, first-served basis. We aim to have a 15-minute drop off and collection at the main entrance subject to availability at the time of your arrival. There is no pre- booking available for these spaces. There is street parking available in the nearby area if you need to park for longer.
7.18.5 Congestion charge
RBH is not in the central London congestion charging zone. But visitors are advised to check the Transport for London website to see if congestion charging will affect their journey to/from the hospital.
7.18.6 Public Transport
RBH is situated in Chelsea, right in the heart of London. The area is well served by the London Underground and bus routes and is close to the amenities of London. Detailed scheduling information can be found on the Transport for London website at www.tfl.gov.uk.
Bus Information
Buses serving the hospital are: Numbers 14, 211, 345 and 414 stop outside the Fulham wing on Fulham Road. Numbers 49 and 211 stop on Sydney Street for our main reception. Numbers 11, 19, 22, 211, 49 and 319 serve the King's Road.
Underground Information
RBH is a ten-minute walk from South Kensington station, which is on the Circle, District and Piccadilly lines. Sloane Square station, served by the District and Circle lines, is a fifteen-minute minute walk from RBH.
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7.19 Visitor accommodation at Royal Brompton Hospital
Accommodation is provided to family members of inpatients. Parents/carers often chose to sleep next to their child on Rose Ward. There is also accommodation in Sydney Street Wing and South Parade (five minutes’ walk from inpatient units).
7.19.1 Reservations
Reservations can be made via the accommodation office on: +44 (0)20 7351 8044.
7.19.2 Who Can Stay
Facilities are intended for short-term use by patients attending outpatient clinics and their immediate family, and inpatients' next of kin. Parents will be issued free accommodation if their child is a paediatric patient, or an adult patient with learning difficulties.
7.19.3 Accommodation Facilities
- Welcome tea and coffee - Welcome toiletry pack - Laundry room - Shared bathroom and kitchen
7.19.4 Reception Opening Hours
The accommodation reception is located on South Parade. Please go to the Fulham wing reception and ask for directions. We are open Monday to Friday 9am to 5pm.
7.19.5 Free Wi-fi Service
Royal Brompton & Harefield NHS Foundation Trust provides a free guest wi-fi service for use by our patients and their relatives. Details of how to register and use the trust’s Wi-Fi are available in outpatients, all inpatient wards and online at https://www.rbht.nhs.uk/patients- visitors/patients/patient-support-services/wifi-service.
8 Patient Movement in the Network
This section will outline key policies and principles in the management and movement of patients in the RBH-CHD Network. All the information in Section 8 is underpinned by agreed policy, governance and framework and is available on either the trust’s intranet or RBH-CHD Network Wed Page. For specific information around access for elective inpatient and outpatient care, please refer to the policy “Waiting list and access policy for elective inpatients and outpatients” available on the RBH intranet or via the CHD Network Management Team
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8.1 Clinical Management
The CHD Working Group have agreed a set of adult and paediatric clinical management guidelines. These guidelines were ratified at the CHD Network Board, and are widely available to every Level 3 Centre, as well as being published on the RBH-CHD Network Web Page. The guidelines are as follows: - Clinical Recommendations for Paediatric Cardiology (Appendix 2) - Management Recommendations for Adults with Congenital Heart Disease (Appendix3)
8.2 Children’s Acute Transport Service (CATS)
The Children’s Acute Transport Service (CATS) is an intensive care 24/7 inter-hospital transport team serving around 50 hospitals in the North Thames, Essex, Herts, Beds, and East Anglia regions in England. The CATS team transport critically ill children at general hospitals without specialist paediatric services, and ensure safe and rapid transfer to a paediatric intensive care unit (PICU). Referral to the CATS team can be made via form (http://site.cats.nhs.uk/wp- content/uploads/2012/08/Referrer_prompt_-sheet.pdf) or by phone (+44 (0)800 085 0003). Advice on when to refer, referral criteria, second opinion and available PICU beds can also be sought from the CATS team over the phone.
8.3 South Thames Retrieval Service (STRS)
The South Thames Retrieval Service (STRS) is an intensive care 24/7 inter-hospital transport team based at Evelina London Children’s Healthcare (ELCH). The team is made up of doctors and nurses from ELCH’s PICU who transport critically ill children at general hospitals without specialist paediatric services to PICU’s in and around London. Referral to STRS can be made via form (https://www.evelinalondon.nhs.uk/resources/our- services/hospital/south-thames-retrieval-service/referral-form-STRS.pdf) or, in an emergency, via phone on either +44 (0)20 7188 5000 or +44 (0)20 7803 9160. Advice can be given over the phone about when to refer, referral criteria, second opinion and available PICU beds. Non-urgent enquires can be emailed to [email protected] or by visiting https://www.evelinalondon.nhs.uk/our-services/hospital/south-thames-retrieval-service/team.aspx and contacting a member of the Consultant Liaison Team.
8.4 Transition
RBH operates a vertical model of care with fetal, paediatric and adult CHD services on one site. Patient flow in this model is aligned with national guidance, specifically with NHS England’s CHD Standards & Specifications. Our current Transition model is summarised in Appendix 1.
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A revised whole-network model, one that reflects future collaborations, is in development. It is expected that each network centre will adopt this model and look to expand and develop on it in their practice, according to preference and resources
8.5 Escalation to Level 1
Where cardiac care cannot be provided to the required level in outreach, referral to RBH is required. This can be planned referral for outpatient diagnostics and consultation, planned day- case assessment or admission, or urgent admission. For any of these options, there is a minimum level of information required from the referring hospital to facilitate smooth handover.
8.5.1 Outpatient Referral
Primary to Level 1 Consultant referral, or secondary to Level 1 Consultant referral should be in the form of a referral letter, and should include the following information: Demographic Information – this essential information should be as comprehensive as possible, and included the patient’s full name and title, DOB, sex (at birth), gender, ethnicity, NHS number, full address, contact details (phone, mobile, e-mail), preferred method of contact, next of kin and full details of their GP practice. Physician Details – this should include full details of the clinic and named consultant the patient is being referred to. Also, full details of the referring practitioner and contact information. Clinical History – information should focus on the presenting complaint, and a detailed clinical history relating to the complaint, a relevant summary of past medical history and management to date. Reason for Referral & Urgency – an explanation as to why the patient is being referred into RBH, and the urgency of the referral. This may include a clear question to be answered, the impact it is having on the patient, completed assessments and / or investigations, risk factors, family and any relevant psycho-social factors.
8.5.1.1 Fetal Cardiology
For fetal cardiology, routine and urgent referral can be made by emailing RBH- [email protected], urgent referrals can be followed up by calling +44 (0)20 7351 8361. Fetal referrals will be allocated to one of four RBH fetal centres as described in section 5.
8.5.1.2 Adult & Paediatric CHD Referral
Currently, outpatient referrals into the CHD service can be sent by post to a named consultant, via a dedicated email ([email protected]) or, in the event of an email system failure, we will accept faxed referrals (+44 (0)20 7351 8028).
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8.5.1.3 Pregnancy & Heart Disease Centre
Email referral letters can be sent to the clinic administrator at RBH, [email protected]. Advice can be given over the phone, +44 (0)20 7349 7748 Patients can choose to have their baby with Chelsea and Westminster Hospital through their local midwife / hospital and local GP. Enquiries can be made via phone, +44 (0)20 3315 6000, or the Chelsea & Westminster self-referral service, http://www.chelwest.nhs.uk/services/maternity- obstetrics/antenatal-self-referral-form.
8.5.2 Level 1 Admission
When RBH is the receiving trust, we will look to ensure that patients are received safely and that the principles of safe practice are applied in every case. Transfers may be complex involving staff, equipment, documents and external agencies such as ambulance services and other providers. Two policies have been approved by RBH to govern the safe transfer of patients:
- Policy for the transfer of patients and their records - Transfer of paediatric patients and their records policy
Both detail the minimum required standards that must be observed in all aspects of adult and paediatric patient transfer and are available on the RBH-CHD Webpage.
RBH will inform the referring hospital whether it can accept the proposed transfer in-line with the following timeframes:
Critical transfer Instant decision Immediate transfer Decision within 30 minutes Non-urgent Decision is case dependent
See Section 6 for details of On-Call Support and Clinical Contact.
8.6 Seven Day Services Standards
The Seven Day Hospital Services Programme was developed to support providers of acute services to deliver high quality care and improve outcomes on a seven-day basis for patients admitted to hospital in an emergency. In November 2018 NHS England & NHS Improvement introduced a new board assurance framework for Seven Day Hospital Services (7DS). This new measurement system replaces the previous self-assessment survey (which was completed twice a year) and consists of a standard measurement and reporting template, which all providers of acute services will complete with self-assessments of their delivery of the 7DS clinical standards. This self-assessment will then be formally assured by the trust board and the completed template submitted to regional and national 7DS leads to enable measurement against the national ambitions for 7DS. Delivery of 7DS is intended to become business as usual for providers.
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8.6.1 Self-Assessment
Of the ten 7DS clinical standards developed in 2013, four were identified as priorities for delivery by the Academy of Medical Royal Colleges: - Standard 2: Time to initial consultant review (first consultant review within 14hrs) - Standard 5: Access to diagnostics (consultant-directed diagnostics) - Standard 6: Access to consultant-led interventions - Standard 8: Ongoing daily consultant-directed review (based on job plans, robust MDT and escalation protocols, local audits)
Self-assessment against the four priority standards will be achieved against a bi-annual board assurance framework (mandated by NHSE and NHSI), using a standard template (self- assessment of our performance against the 7DS clinical standards supported by local evidence), which is then presented to and assured by the board before results are submitted to regional and national 7DS teams. The measurement template lists the four priority 7DS clinical standards (standards 2, 5, 6 and 8 as above). It also captures detail on 7DS in urgent network specialist services with regard to the four priority 7DS clinical standards: - Hyper acute stroke (not applicable to RBH) - Paediatric intensive care - STEMI heart attacks - Major trauma (not applicable to RBH) - Emergency vascular services Progress against the six remaining 7DS standards for continuous improvement will not be formally measured through the collection of data or formal self-assessments, however they remain important and are enablers for the delivery of the four priority clinical standards. Therefore, the measurement template includes a narrative section for us to summarise our progress against the remaining six standards (1,3,4,7,9 and 10).
8.6.2 RBH Board Assurance
The board-assured assessments of 7DS delivery will feed into local and national accountability frameworks. The board is required to give formal organisational assurance of the self-assessment of 7DS delivery contained in the completed measurement template and any supporting paper before it is formally submitted to regional and national 7DS teams. These will be internal metrics available via national frameworks however, board assured self- assessments can be shared in the network if requested.
9 Quality and Governance
Following the publication of NHS England’s CHD Standards and Services, each Level 1 CHD provider must formalise their referral networks and monitor compliance against the new standards with the aim of improving patient care and access. In this section, key quality channels and
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mechanisms will be described that allow the Network Management Team to deliver against the standards, and implement good governance and practice across the operational delivery network.
9.1 MDT Meetings
Multidisciplinary care at RBH is an integrated team approach to health care in which medical, nursing, and allied health care professionals consider all relevant treatment options and develop collaboratively an individual treatment plan for each patient. There are many CHD MDT Meetings that support the delivery of CHD Services at RBH; the Adult CHD MDT Meeting and the Joint Cardiac Conference (JCC) for paediatric patients (detailed in the following section), Electrophysiology MDT meetings (adults and paediatrics, weekly), Inherited Cardiac Conditions MDT meetings (adults and paediatrics, monthly), and cardiomyopathy flag MDT meetings (weekly). There is also a weekly fetal governance meeting to allow collective discussion of complex fetal patients. In addition, fetal cases are discussed in the fetal medicine MDT meetings at our linked obstetric units. Full operational guidance around the Adult CHD MDT and the JCC MDT Meetings can be found in the Congenital Heart Disease Multidisciplinary Team Meetings Terms of Reference on the RBH- CHD Webpage.
9.1.1 Adult CHD & JCC MDT Meetings - Timing and Venue
The Adult CHD MDT Meeting will take place weekly on Friday mornings at 08:00 in the Cardiology Seminar Room, Chelsea Wing, RBHT. The JCC Meeting will take place 3 times per week on Monday, Tuesday and Thursday mornings at 08.00 in the Cardiology Seminar Room, Chelsea Wing, RBHT. The Tuesday meeting includes discussion of fetal cases and the Thursday meeting is a pre-surgical meeting including cases planned for surgery the following week. A separate fetal governance meeting for the fetal cardiology team follows on from the JCC. The day and time of meetings should be consistent and regular to ensure quoracy. The duration of the meetings will be determined by each CHD MDT based upon the number and complexity of cases requiring discussion. Any time not used for case discussion may be used for educational purposes or discussion of other relevant hospital related issues.
9.1.2 Adult CHD & JCC MDT Membership & Quoracy
Membership of the RBHT CHD MDT meeting may comprise medical staff, echocardiographers, nursing, allied health, pharmacist, psychosocial professionals, other supportive care services providing clinical services in relation to delivery of care. Each MDT meeting will have a Clinical Chair who should be a medical / surgical consultant from a relevant specialism.
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Each Royal Brompton Hospital MDT will have a core membership, and a list of key attendees that will ensure assembly of a quorate group.
9.1.2.1 Adult CHD MDT Meeting Membership
Quoracy - Clinical Chair Dr Isma Rafiq (or designee) - 2 ACHD specialists - 1 Adult Imaging Specialists - 1 Cardiac Surgeon
Membership - All consultants in ACHD including cardiologists and surgeons - Paediatric consultant staff - Junior medical staff working within the ACHD clinical group - ACHD & Transition Clinical Nurse Specialists - Imaging consultants from ECHO, CMR and CT. - AICU and Anaesthetic staff
Other specialists as required for individual cases - Adult Airway Team - Adult Intensive Care - Specialised Adult Anaesthesia - Adult Surgery - Adult Nephrology - Adult Gastroenterology - General Adult Cardiology - Paediatric Airway Team - Paediatric Cardiac Surgery - Vascular Services including Surgery and Interventional Radiology
9.1.2.2 JCC Meeting Membership
Quoracy - Clinical Chair (rotational) - 2 Paediatric CHD specialists - 1 Paediatric Imaging Specialist - 1 Paediatric Cardiac Surgeon
Membership
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- All consultants in Paediatric CHD including cardiologists and surgeons - Fetal Cardiologists - Paediatric consultant staff - Junior medical staff working within the Paediatric CHD clinical group - Paediatric CHD & Transition Clinical Nurse Specialists - Imaging consultants from ECHO, CMR and CT. - PICU and Anaesthetic staff - Echocardiographers - Paediatricians with Expertise in Cardiology - Paediatric electrophysiologists
Other specialists as required for individual cases - Paediatric Airway Team - Paediatric Intensive Care - Specialised Paediatric Anaesthesia - Paediatric Surgery - Paediatric Nephrology - Paediatric Gastroenterology - General Adult Cardiology - Adult Airway Team - Adult Cardiac Surgery - Vascular Services including Surgery and Interventional Radiology
9.1.2.3 Fetal Governance Membership
- 4 Fetal Consultants - 4 Fetal Clinical Nurse Specialists - Fetal Cardiology Fellows - Paediatric Cardiology Trainees (all welcome)
9.1.2.4 Support staff
Support staff members may be required to assist with meeting implementation, for example administrative assistants, imaging support staff (e.g. Picture, Archiving & Communications Administrators) and IT.
9.1.3 Discussion Outcome
Treatment and management recommendations from the meeting discussion will be documented on appropriate proforma’s or medical record continuation sheets which must be made available to the referring clinician and inserted in the patient's electronic health record.
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The referring clinician or designee is responsible for discussing the meeting recommendations with the patient / family / carer and developing the treatment plan which takes into account the patient preferences. This plan is to be made available to relevant team members, the patient’s GP (where appropriate) and noted in the patient’s clinical health record.
The report from each case will be published in the electronic patient record. In addition, the electronic MDT database will be updated on a weekly basis.
9.2 Paediatric Ward Rounds
RBH has daily morning ward rounds for paediatric cardiology seven days a week. Monday to Thursday is always led by the lead paediatric consultant cardiologist (or designee) for the ward. On Friday, Saturday and Sunday, the ward round is led by the on-call paediatric consultant cardiologist. The round will consist of a lead consultant, junior doctors and nurses.
9.2.1 PICU / PHDU Ward Rounds
RBH Paediatric Intensive Care Unit (PICU) has twice daily ward rounds; a 07:30 surgical round, and a 14:00 intensivist round, seven days per week. Both are consultant led, have junior doctors and nurses in attendance, but also advanced nurse practitioners. RBH PHDU also has twice daily ward rounds with the same membership as the PICU ward rounds. Timing of the Paediatric High Dependency Unit rounds will be set by the lead consultant.
9.2.2 CARE
There is a weekly Care & Liaison Meeting, referred to as CARE. Patients with complex needs, identified on admission and seen in rounds, will be discussed in this forum. Attending this meeting will be a consultant (lead), junior doctors, nurses, and as required, advanced nurse practitioners, pharmacy, safeguarding, play specialists, clinical nurse specialists, dietetics, physiotherapy, psychology and family liaison. This is a formal meeting, the output of which will feed into a patient’s care pathway.
9.3 Adult Ward Rounds
RBH adult ward rounds occur daily and are always consultant led. However, when compared to our paediatric CHD patients, adult CHD patients are less frequently inpatients. Because of this, there is no dedicated CHD specialist on the ward to lead weekday rounds. Instead, the responsibility of the rounds will fall to the on-call adult CHD consultant and will occur daily.
9.3.1 AICU / HDU Ward Rounds
AICU and Adult HDU have ward rounds three times per day. Every round is consultant intensivist led with junior doctors, nurses and advanced nurse practitioners in attendance. There will be a morning round, a late afternoon round that must conclude before 20:00, and an evening round
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after 21:00. If needed, a fourth round will be made at night driven by the consultant intensivist on the unit.
9.4 Image Sharing
Image sharing in the RBH-CHD Network is currently dependent on the Image Exchange Portal, NHS.net email, fax and secure courier. Non-urgent image review in the network will be triggered by consultant-to-consultant communication / request for advice. Urgent review of imaging will always be in conjunction with a request for advice via the Registrar on-call (see Section 6).
9.4.1 Image Exchange Portal
The Image Exchange Portal (IEP) provides a connection to all the trusts in the RBH-CHD Network. The RBH IEP can be accessed via the trust’s intranet in the form of an IEP request, or by contacting RBH Imaging Clerks directly in the RBH PACs Team (based on our Harefield site). Requests are processed via nww.iepservice.nhs.uk. During working hours, the transfer service via IEP is provided by imaging clerks, and for cases with technical issues the PACs Team. Outside of working hours transfer service via IEP is provided by on-call radiographers.
9.4.2 Other Channels for Image Exchange
Paediatric and adult clinical imaging can be transferred via email or fax using the contact details outlined in Section 6. Where the image is multi-layered and / or shows a moving functional aspect, files can be saved onto secure data storage devices and securely couriered to a named clinical contact at RBH.
9.5 Board and Subcommittee Meetings
The RBH-CHD Network Board will be accountable to the Medical Director of Specialised Services, NHS England, London. The Board will also have intra-hospital accountability to Dr Vias Markides (Divisional Director, Chair of Adult Quality & Safety Meeting, Heart Division) and Dr Jan Till (Co- Director of Paediatrics, Chair of Paediatric Quality & Safety Meeting). The RBH-CHD Network Chair will oversee the ratification and submission of key strategic work in the operational delivery network. The network board will provide strategic operational direction to the network management team. It will also have oversight of the five Network Sub-Committees illustrated above. Each committee will act as a driver for delivery of strategic objectives outlined in the RBH-CHD Strategic Vision. Each committee Chair will hold the Network Management Team to account in the delivery of agreed actions and ensure progress is captured and tracked on an agreed quality improvement plan that will feed into the network boards overarching improvement initiatives.
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The structure, and lines of accountability, are illustrated below.
Adult Quality & Safety Committee & Medical Director Paediatric Quality & Specialised Safety Committee Services, London NHS England
CHD Network Board
Quality, Pathways & Patient Technology Safety & Transition Protocols Experience Audit
Full details of the Network Board can be found in the Royal Brompton & Harefield Congenital Heart Disease Network Board Terms of Reference.
9.6 Approval Process: Network Documentation & Patient Information
The RBH-CHD Network Management Team are responsible for the general administration of clinical and non-clinical network documents and policies. The team will adopt simple development and ratification processes as outlined in this section
9.6.1 Development and Ratification
Network documentation, excluding patient information, for the RBH-CHD Network will be drafted and peer reviewed by CHD Clinical Working Groups or CHD Non-Clinical Working Groups as needed. An RBH-CHD Network Sub-Committee may identify the need for documentation, and may submit certain documents to the RBH-CHD Network Board for publication. If the scope of the document falls outside of network governance and there are wider ranging implications for RBH, documents must be ratified by the hospital’s Quality & Safety Committee. From here, documents can be further escalated to the Governance & Quality Board if required. All documents ratified at network level will be included in a notes section for the relevant Quality & Safety Committee (Adult or Paediatric). These documents will mostly be included for information only, and usually not scheduled for discussion.
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The process for patient information differs slightly to that for network documentation in that there is a need for patient feedback and review of comments. The RBH-CHD Network Management Team will adopt guidance issued by the Brompton Fountain Charity (Guidelines for producing patient information literature - found here) and seek to engage relevant patient groups in conjunction with the charity. Time lines for feedback and consultation will be set by the working group developing the document. These will likely be dictated by the size of the document and the volume of feedback required. Once adequate patient feedback has been canvased and incorporated where possible, the route of approval will mirror that of network documentation. The process for both development and ratification for both network documentation and patient information is summarised in the following chart.
10 Research
Patients are central to the work of RBH and the hospital’s Research & Public Involvement document sets out our strategy for ensuring effective patient and public engagement and participation. RBH is a research organisation that focuses on involving patients and members of the public in developing, designing and implementing research. RBH ensures that the research is:
- relevant to the people it is trying to help - beneficial in terms of delivering meaningful outcomes for patients - conducted in a way that is sensitive to the needs of the participants and their friends/family through better patient information, recruitment processes and general management of the project and the project outcomes.
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The full strategy is available on the RBH Website at https://www.rbht.nhs.uk/research/public- involvement-our-research.
11 Audit
The RBH-CHD Quality & Safety Teams ensure robust processes are in place for the tracking and periodic submission of local and national audit. Local audit is registered on the hospital’s CIRIS for Healthcare System. All audits on this system will have an associated abstract and clear outcome reported. Completed audits for RBH will be published in the annual report, with an eventual aim of opening this to include network audits. RBH also submits to the National Congenital Heart Disease Audit (NCHDA) which is hosted by the National Institute for Cardiovascular Outcomes Research. Summary comments around our most recent ACHDA validation will be included in the RBH-CHD Network annual report, as will outcome data around surgical and interventional activity.
12 Patient Experience
The patient journey is central to the delivery of CHD services in the operational delivery network. The RBH-CHD Strategic Vision (available on the RBH-CHD Webpage) highlights core values that ensure our teams remain focused on patient experience, they are: - Place the patient at the heart of everything we do. - Focus on improving the value proposition for our patients and their families. - Listen and learn to allow us to evolve in line with the changing landscape of CHD medicine and patient need. - Evidence improvement with the use of robust patient reported experience measures. This section will describe key offerings and contacts for our patients in the CHD Network.
12.1 Patient Advice & Liaison Service (PALS)
The patient advice liaison service (PALS) is a confidential service offering support and advice to patients, families and carers. The PALS Office is onsite at RBH near the Sydney Street reception. We are available, Monday- Friday, 9am-4pm, excluding bank holidays. Address: PALS Royal Brompton Hospital London SW3 6NP T: +44 (0)20 7349 7715 E: [email protected]
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Our PALS online contact form can be found at: https://www.rbht.nhs.uk/patients- visitors/patients/patient-support-services/patient-advice-and-liaison-service-pals/contact-pals.
12.2 Support for In and Out Patients
RBH provides a service to adult patients, paediatric patients and their families to support them living with CHD these include:
12.2.1 Safeguarding and Learning Disabilities Care
People with learning disabilities, autism or both find it much harder than other people to access assessment and treatment for general health problems. Within the Trust they are amongst the most vulnerable population groups. RBH has a Safeguarding Lead who also is the Lead for Learning Disabilities Care. The Safeguarding and Learning Disabilities Lead is the first port of call for any support required and will support staff in devising a care plan to meet the needs of the patient with a learning disability. They also advise on pre-admission planning, communication, use of hospital passports and making reasonable adjustments that may be needed to ensure that high quality care is given to all patients. The Safeguarding and Learning Disabilities Lead also liaises with any community teams involved in the care of a patient with a learning disability and engages their support to facilitate a timely and safe discharge from hospital. Before providing clinical treatment for people with learning disabilities, healthcare staff must ensure that consent is sought. Treatment that is thought to be in his/her best interest is provided in accordance with the Mental Capacity Act 2005 guidelines. The Safeguarding and Learning Disabilities Lead is on hand to provide support with regards to capacity assessments and best interest decisions. Safeguarding is also a key priority within paediatrics. The Trust is committed to ensuring that we are fulfilling our duty of care and complying with local and national policy and legislation to prioritise the safeguarding of children and young people and promoting their wellbeing. The dedicated Safeguarding Children Team work closely with RBH colleagues and inter-professionally to ensure that children, young people and family’s needs are considered holistically, with coordinated partnership working with community teams ensuring safe discharge and support or safeguarding referrals facilitated where such need is identified in line with Children Act 1989 and 2004.
Contact details: Frank Butau Trust Lead for Safeguarding Adults & Learning Disabilities Royal Brompton & Harefield NHS Foundation Trust Sydney Street, London SW3 6NP T: +44 (0)20 7351 8121, Extension: 84195 M: +44 (0)7484 037301
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Lorna Waite Named Nurse for safeguarding children and young people Royal Brompton & Harefield NHS Foundation Trust Sydney Street, London SW3 6NP T: +44 (0)20 7351 8121, Extension: 82903 M: +44 (0)7896 220697
Hannah Wallace Paediatric Social Worker Royal Brompton & Harefield NHS Foundation Trust Sydney Street, London SW3 6NP T: +44 (0)20 7351 8121, Extension: 84289 M: +44 (0)7484 926928
12.2.2 Parents with Learning Difficulties
Primarily we identify parents who may need additional support to understand the treatment options for their child and be supported to make decisions by:
- Self-identification; we encourage anyone to feel comfortable to share their specific learning difficulty with us. - Shared by Health visitor, midwife or General Practitioner for example when child is referred to RBH. - Assessment of the professional interacting with parent(s) and perceiving addition needs to foster understanding of their child’s condition and treatment options.
Any member of staff who is given such information will share it with the appropriate members of the MDT once the individual has agreed with what we as a team can offer them to help. Key staff to help:
- Consultant - Clinical nurse specialist - Named nurse (if inpatient) - Psychology - Safeguarding (potentially if social services support is needed for ongoing care) - Play Team - Family liaison
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Plans will be made for the individual according to their and their child’s needs. Parent facilities are as normal for any parent. However, a person with learning difficulties may need added support to locate accommodation of the kitchen / sitting room facilities. They will be given bespoke help to ensure they are well supported whilst their child is in hospital. Specialist Nurses will liaise with community team, primarily health visitor, to ensure that there is seamless support long term.
12.2.3 Adult Clinical Psychology
The adult clinical psychology service is a cross-site provision that delivers psychological assessment and treatment services to patients, carers and families regarding psychological aspects of care. There is a 1.0 WTE Consultant Clinical Psychologist for adult cardiac patients who will see RBH adult CHD patients. Contact details: Adult Clinical Psychology Services Royal Brompton & Harefield NHS Foundation Trust Sydney Street, London SW3 6NP T: +44 (0)20 7351 8121, Extension: 88060
12.2.4 Paediatric Clinical Psychology
RBH has 2.0 WTE clinical psychologists working with child patients with CHD and their families. Referrals are accepted from anybody (10% are self-referrals), and offered to the patient and their parent(s), siblings and extended family (if appropriate). Work includes offering counselling to parents of a very sick child, as well as working with the child/young person themselves to promote their coping and aim to support them to live as ordinary lives as possible in the context of having CHD. Psychometric assessments can be offered to identify any possible learning difficulties and psychological therapies are utilised to assess and offer interventions for challenges which may or may not be due to the cardiac condition such as anxiety around invasive procedures, behaviour or mood challenges. The psychologists work closely with the rest of the child and young person’s multidisciplinary team at RBH (or Harefield Hospital) and in Networks and other statutory and voluntary community services. The psychologists regularly attend MDT meetings and offer consultation, teaching and training where appropriate. Contact:
Lead Clinical Psychologist Royal Brompton Hospital London SW3 6NP
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T: +44 (0)20 7352 8121, Extension: 84130
12.2.5 Paediatric Play & Therapy
The Paediatric Play Team is staffed on a 7.35 whole time equivalent basis. They cover Rose Ward, Paediatric Intensive Care and Paediatric Outpatients. The Paediatric Play Team run a 7 day a week service for the inpatient unit. Through play, The Hospital Play Specialists and Play Assistants are able to provide the children with a wide range of creative and social activities and play opportunities, whether this is in the Playroom, Play Area or at the bedside. The Paediatric Play Team are there to support the children throughout their hospital visit or admission, providing a range of services:
- Providing developmentally appropriate play opportunities, designed to engage and inspire the children. - Preparation and distraction techniques prior to and post surgeries, treatments and procedures. - Developmental Play Programmes for long term patients. - Specific play programmes to children with needle anxiety. - Routines, Plans and social stories to engage children with Autistic Spectrum Disorder. - To help provide the best possible hospital experience. Contact: Play Service Manager Rose Ward Royal Brompton Hospital London SW3 6NP T: +44 (0)20 7352 8121, Extension: 82026
12.2.6 Family Liaison
The family liaison service, based at RBH, is a non-medical / nursing team that can support you from your child’s admission through to discharge. The team can act on behalf of a patient or patient’s parents if permission is given. They can cross- refer to other services in the hospital and frequently link with the safeguarding, psychology and fetal teams. They will also listen to concerns and advise where appropriate, aid in completing forms and documentation, help with accommodation arrangements, and link with external agencies and other hospital staff. Address: Family Liaison Team Rose Ward
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Royal Brompton Hospital London SW3 6NP T: +44 (0)20 7352 8121, Extension: 88588 M: +44 (0)7484 936290
12.2.7 Breastfeeding & Expressing Support Team (BEST)
RBH is able to support and inform new mothers if they choose to breastfeed or express via the BEST. There are dedicated facilities available for expressing on Rose ward or on intensive care. The BEST can also provide a pump that can be taken to the parent accommodation.
12.2.8 Chelsea Community Hospital School
The school team at RBH enables children to access education while they are in hospital. They provide students with a broad and balanced curriculum – either in the classrooms, or at a child’s bedside (on both Rose Ward and PICU) – and can personalise provision according to students’ individual needs. The also team establishes crucial links with children’s home schools in order to provide continuity of education, particularly essential for students who may spend many weeks of their school life in hospital. For children in hospital, the schoolroom can offer a sense of normality; children can interact with their peers, take part in a range of academic and creative activities, have some one- to-one tuition or even sit exams if they need to. Address: Chelsea Community Hospital School Royal Brompton Hospital London SW3 6NP T: +44 (0)20 7352 8121, Extension: 82415 F: +44 (0)20 7351 8505 E: [email protected]
12.2.9 Bereavement Services
RBH bereavement service offers one-to-one support for relatives and those close to patients who have died in the hospital. The service offers practical advice and support about what needs to be done during this difficult time. The bereavement officer is a member of our staff who can offer help to bereaved families and friends. The bereavement officer is notified of all deaths in the hospital and will meet patient family and friends as soon as possible to discuss arrangements and to advise on the practical steps that need to be taken.
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In the paediatric service, all families are offered an appointment with the patient’s cardiologist and other appropriate / nominated staff members (e.g. PICU consultant, CNS, ANP) approximately 6 weeks after death to discuss post-mortem results etc. All families are also contacted at this time by the psychology team to offer to support the family to access counselling either locally or at RBH. Address:
Sarah Taylor Bereavement Officer Royal Brompton Hospital London SW3 6NP T: +44 (0)20 7349 7715
12.2.10 Patient Information
All patient information regarding RBH, services / infrastructure and accessing the hospital (Section 7), and information around clinical conditions and tests, are available on the RBH-CHD Network webpage. This information supplements our 24/7 CNS Service that will advise and support our CHD patients.
12.2.11 Brompton Fountain
The Brompton Fountain is a charity supporting children with serious, often life threatening, medical conditions who are under the care of RBH. They provide vital support and resources, medical equipment and services which are not normally supplied by the NHS. They work closely with the hospital teams within the paediatric intensive care unit and children's wards. They fundraise to improve the quality of life for RBH’s young patients and their families. The team have first-hand experience of caring for a child with a serious illness and are able to offer support to these children and their families before and during admission. Many RBH patients have life limiting conditions and will require frequent admissions and often long stays in hospital for surgery and on-going treatment for the rest of their lives. The Brompton Fountain also offers support to patients and families after discharge from hospital with information around helping families at home within the community.
12.3 Contact Information
Contact information for our 3 core CHD CNS Teams are as follows.
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12.3.1 Fetal CHD Contact and 24/7 Support
The following information can be used by fetal CHD patients for advice, guidance and out-of-hours emergency contact. Lead Nurse Paediatric Congenital Heart Disease Services Royal Brompton and Harefield Hospitals NHS Foundation Trust Sydney Street London SW3 6NP M: +44 (0)7794 020390 Susana De Sousa (Fetal CNS) M: +44 (0)7790 375856 Sarah MacCarthy-Morrogh (Fetal CNS) M: +44 (0)7969 660820 Julieta Lumanisakio (Fetal CNS)
12.3.2 Paediatric CHD Contact and 24/7 Support
The following information can be used by paediatric CHD patients for advice, guidance and out-of- hours emergency contact.
Address:
Lead Nurse Paediatric Congenital Heart Disease Services Royal Brompton and Harefield Hospitals NHS Foundation Trust Sydney Street London SW3 6NP
E: [email protected] T: Paediatric CHD: +44 (0)330 128 7727 (Clinical Nurse Specialist Team)
For out-of-hours emergency:
T: +44 (0)330 128 8121, ask for the on-call registrar for paediatric cardiology, who will be able to contact the Paediatric CHD Consultant on-call.
12.3.3 Adult CHD Contact and 24/7 Support
The following information can be used by adult CHD patients for advice, guidance and our of hours emergency contact.
Address:
Lead Nurse Adult Congenital Heart Disease Centre
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Royal Brompton and Harefield Hospitals NHS Foundation Trust Sydney Street London SW3 6NP
E: [email protected] T: Adult CHD: +44 (0)330 128 8764 (Clinical Nurse Specialist Team) T: PAH Office: +44 (0)330 128 8362 (Pulmonary Hypertension Team)
For out-of-hours emergency:
T: +44 (0)330 128 8121, ask for the on-call cardiology registrar for adults, who will be able to contact the ACHD Consultant on-call.
12.3.4 Inherited Cardiac Conditions
The following information can be used by adult and paediatric Inherited Cardiac Conditions patients for advice, guidance and out of hours emergency contact. Address: Lead Nurse Inherited Cardiac Conditions Royal Brompton and Harefield Hospitals NHS Foundation Trust Sydney Street London SW3 6NP T: +44 (0)20 7352 8121, extension: 82205 For out-of-hours emergency:
T: +44 (0)330 128 8121, ask for the on-call cardiology registrar for adults if you are an adult patient, or the on-call cardiology registrar for paediatrics if you are a paediatric patient
12.3.5 Palliative Care
Linda Freeman Therapies / Palliative Care Team Royal Brompton and Harefield Hospitals NHS Foundation Trust Sydney Street London SW3 6NP
T: +44 (0)20 7352 8121, extension: 84136 (Mon-Fri, 8:30am-4:30pm)
12.3.5.1 Specialist Paediatric Palliative Care
Great Ormond Street Hospital Palliative Care Team Great Ormond Street Hospital for Children NHS Foundation Trust
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Great Ormond Street Holborn London WC1N 3JH T: +44 (0)20 7829 8678 (Mon-Fri, 9:00am-5:00pm)
PATCH (Paediatric Palliative Care) Royal Marsden Hospital NHS Foundation Trust 203 Fulham Road London SW3 6JJ T: +44 (0)20 8661 3625 (Mon-Fri, 9:00am-5:00pm)
12.3.5.2 Specialist Adult Palliative Care
Adult Palliative Care Team Royal Marsden Hospital NHS Foundation Trust 203 Fulham Road London SW3 6JJ
T: +44 (0)20 8642 6011 (Mon-Fri, 9:00am-5:00pm)
12.3.5.3 Other organisations that offer support
British Heart Foundation (www.bhf.org.uk) T: +44 (0)20 7554 0000 (Mon-Fri, 9am-5pm)
Macmillan Cancer Support (www.macmillan.org.uk) T: +44 (0)808 808 0000 (Mon-Fri, 9am-8pm)
British Lung Foundation (www.lunguk.org) T: +44 (0)3000 030 555 (Mon-Fri, 9am-5pm)
12.4 Feedback
In April 2019 the CHD specific patient reported outcome measures programme was suspended due to the withdrawal of the coordinating consultancy firm. The RBH-CHD Network Management Team and RBH-CHD Board are therefore reliant on the Friends and Family Test.
12.4.1 Friends and Family
The Friends and Family Test was introduced by NHS England as a key measure to improve patients’ experiences of care across the NHS. All hospital trusts are mandated to ask all inpatient
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and day-case patients: “How likely are you to recommend our ward / clinic to friends and family if they needed similar care or treatment?” The Friends and Family Test (FFT) provides a simple headline metric which, when combined with follow-up questions, is a tool to ensure transparency, celebrate success and make improvements where necessary to ensure that patients have a positive experience of care. Results of the test are published monthly on the NHS England and NHS Choices websites. The Trust collects data by various methods; paper questionnaires given to all patients on the day of discharge, online via tablets, or as a response to a text message sent 48 hours post discharge. The FFT target score first set by the Department of Health was 15%, this was increased to 25% in April 2014 and the Trust has managed to achieve and exceed these targets. As from 1st January 2015 the FFT target increased to 30% and this has been achieved consistently. There is an acknowledged limitation when using this data, it is not possible to extrapolate CHD patient specific feedback. However, adult and paediatric data relating to the areas and services of the hospital CHD patients frequent the most can be isolated. Some CHD specific verbatim comments can also be collected in this data set. FFT recommendation rate assigned to those identified services and areas will be used as an interim gauge for CHD patient feedback until the national CHD patient feedback programme is relaunched. Findings will be published in the RBH- CHD Network Annual Report and made available on the RBH-CHD Network Webpage.
13 Network Audit and Review
The RBH-CHD Network will champion continuous quality improvement and aim to realise its strategic vision. The channel for delivery will be members of the RBH-CHD paediatric and adult teams, with oversight and accountability from our board and sub-committee structure. As well as quality improvement working groups, the RBH-CHD Network Management Team will also produce three key documents annually to evidence progress and forward planning. They will be an audit against key quality indicators as identified by the Quality Surveillance Team (NHS England), an annual report outlining key achievements over the financial year, and a business plan (including work plan) detailing areas of focus and specific targets to attain in the coming 12 months.
13.1 Annual Audit Against QIs
NHS England’s CHD Standards and Services were used as a template by the NHS England Quality Surveillance Team (QST) to develop a set of Quality Indicators (QIs). The national guidance was reviewed by a Clinical Reference Group, sent out for consultation, edited in-line with feedback and published in 2019 (the full QIs for a CHD network, Specialist Children’s Surgical Centre (SCSC) and Level 1 ACHD Centre are available on the RBH-CHD Webpage. These QIs will be used as a framework for gap analysis against the RBH-CHD Operational Delivery Network. The output from this exercise will be published in RBH-CHD Annual Report and will also help inform the work plan for the preceding year. The QI audit will also be fed back to the QST in the form of a self-declaration, the expected submission date to be April / May each year.
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13.2 Annual Report
In April, every year, the RBH-CHD Network Management Team will draft an Annual Report for ratification at the RBH-CHD Network Board. Following approval, the report will be published on the RBH-CHD Webpage and disseminated via email to network members. The aim of the report is to outline achievements and challenges over the past 12 months. It will also encompass quality performance metrics around CHD provision in RBH as follows: - Incidents - Clinical Outcomes - Operator Volumes - Multidisciplinary Meeting Performance (again the ToRs) - Patient Feedback - Complaints - Educational Delivery
13.3 Business Plan
The purpose of this document is to highlight the work being carried out by RBH, and network member hospitals, to develop the RBH-CHD Network. It will bring into focus activities that are due to be delivered in the coming year and so enable the RBH-CHD Network to move towards full alignment with national standards as well as deliver on strategic goals agreed at the network board. The plan will consist of the RBH-CHD Strategic Vision, the structure (operational and governance) that will allow us to achieve our goals, network objectives, ongoing collaborative work under the King’s Health initiative, and a work plan.
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14 Appendix 1 – Transition Pathway
Note
Transition is a Nurse-Led service at RBH. Developments in
this area will be coordinated by the Lead Nurse for CHD and agreed by the Network Transition Sub-Committee
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15 Appendix 2 – Clinical Recommendations for Paediatric Cardiology
15.1 Introduction
The following are management recommendations for children with congenital heart disease. They are not proscriptive but meant as guidelines, recognizing that individualization is necessary in many cases. They do not include every type of cardiac disorder but address the more common lesions and other common cardiac non-congenital conditions.
15.2 Nomenclature
15.2.1 Basic Investigations
This refers to a clinical examination, plus or minus ECG and Chest X-ray (all tests at the cardiologist’s final discretion for example: consideration might be given to not performing routine Chest X-Rays at post-op visit if pre-discharge X-Ray satisfactory). For all cyanotic patients this would also include oxygen saturation at time of clinic assessment, plus haemoglobin and haematocrit at discretion of cardiologist.
15.2.2 Other Investigations
CT and Magnetic resonance imaging For more complex aortic arch anomalies and for older children with complex anatomy, CT or CMR may be considered if inadequate imaging by echo. CMR is also becoming the investigation of choice in the assessment of RV function for example after repair Tetralogy of Fallot while CT adds to our understanding of coronary anatomy. Exercise testing MVO2 exercise testing can be a helpful objective measure of cardiovascular efficiency, and is particularly useful in serial follow-up of patients and for timing of interventions such as pulmonary valve replacement following Tetralogy of Fallot repair. Standard exercise testing or Thallium scanning is preferred in the investigation of ischaemia. Karyotyping This should be performed in all conotruncal lesions and those with a right aortic arch looking for 22q11 deletion, as well as lymphocyte subsets before surgery in these patients (if awaiting karyotype results) to decide if irradiated blood products are required (see 22q11 guidelines on Trust intranet). and indeed, there should be a low threshold for other complex lesions or where there are lesions specific to particular syndromes, such as subaortic stenosis and Williams Syndrome, etc.
15.3 Timing of First Follow-Up Visit
In general terms the first follow-up visit after surgery should be at 2-3 weeks after discharge mainly to check for pericardial effusion. If clinics are full, the effusion check should be as a day case with
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a formal outpatient appointment at a later date. After a catheter intervention the first follow-up is usually at 3 months although this may be sooner at the Cardiologist’s discretion.
15.4 Anomalous Left Coronary Artery from the Pulmonary Artery
Preoperative studies – All require echo prior to surgical re-implantation. CT or cardiac catheter may be performed if the diagnosis by echocardiography is unclear however caution must be exercised if ventricular function is poor. Postoperative studies
Myocardial Basic Echo Perfusion pre discharge * * 2-3 weeks * * 3 months * * 12 months * * every 1-2 yrs * * individualize
An exercise test is likely to be useful in older children or adolescents, as well as stress echo or stress CMR if any symptoms or concerns about coronary ischaemia. Myocardial perfusion scan (nuclear medicine or CMR) may be indicated if there are concerns about function on echo.
15.5 Anomalous Pulmonary Venous Drainage (total).
Preoperative studies Echo only with typical findings. + Angiography, CMR, or CT angiography may be necessary if unable to see all 4 pulmonary veins by echo and there is a possibility of mixed drainage. Postoperative studies
Basic Echo Holter pre discharge * * 2-3 weeks * * 3 months * * 6 months * * 1 year * * * *preschool, and at ages 10 & 13 & at 3-5 years * individualise transfer to ACHD care (1)
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+ = Further investigations individualized based on echo result Angiography, CMR, or CT angiography may be necessary if there is any evidence of pulmonary venous obstruction. At age 10 consider as well as ECG & echo to perform exercise testing, repeat Holter, MRI and screening cholesterol.(1)
15.6 Aortic Valve Stenosis
Preoperative studies All require echo prior to intervention. Yearly follow-up for those > 2 years, 6 monthly < 2 years. If > 8 years of age then exercise ECG may be considered if estimated peak to peak gradient is > 40 mmHg but less than 50 mmHg, unless there is ischemia on resting ECG. The spectral recordings resulting from Doppler interrogations of aortic stenosis reflect the highest gradients within the jet. This is frequently referred to as ‘peak instantaneous gradient’. Doppler estimates of the aortic peak gradient calculated using the simplified Bernoulli’s formula generally overestimate severity when compared with catheterization peak-to-peak gradients. The mean gradient on echo is similar to the ‘peak to peak’ gradient and should be routinely recorded.. Indications and type of intervention Neonatal Period (< 1 month age): Balloon dilatation to be considered unless otherwise contraindicated by valve morphology. JCC to decide on strategy – surgery may be preferred. If there is associated coarctation of the aorta, balloon dilatation followed by surgical repair of coarctation or both lesions treated by balloon dilation. Outside Neonatal Period (>1 month): Those with poor LV function balloon angioplasty, decision not based on echo gradient. In the presence of normal LV function: intervention if estimated peak to peak gradient > 50mmHg, presence of left ventricular hypertrophy, presence of repolarisation abnormalities on ECG, presence of symptoms or if there is exercise induced ischemia. Postoperative studies
Basic Echo Exercise Treadmill pre discharge * * 3 months * * 1 year * * every 1-2 years * * ? > 7 years
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Early follow up after 2-3 weeks following surgical intervention or if poor function or significant aortic regurgitation at time of discharge following catheter intervention.
15.7 Aorto-Pulmonary Window
Preoperative studies Diagnosis is based on echo prior to surgery however a CT angiogram or catheter may be required to image the coronary arteries if the origins are unclear, or if there are concerns regarding pulmonary vascular resistance. It may be possible to close small A-P windows by catheter technique. Postoperative studies
Basic Echo Pre discharge * * 2-3 weeks * * 6 months * 18 months * * every 2-3 years * individualize
15.8 Atrioventricular Septal Defect
Preoperative studies All patients should have an ECG, Chest X-ray and Echo prior to surgical intervention plus thyroid function studies in those with Trisomy 21 (usually performed locally). Cardiac catheterization is reserved for those cases with pulmonary hypertension older than 9 months of age at presentation, or if there are other concerns that cannot be resolved by Echo. Patients with pulmonary hypertension should be electively repaired in the first 3-5months of life, as delay beyond this is disadvantageous. If there is associated coarctation of the aorta, repair coarctation first (± pulmonary artery banding). Patients with an ostium primum ASD in isolation (+/- small restrictive ventricular component) should be electively repaired at about 2-4 years of age, unless complicating features such as significant atrioventricular valve regurgitation dictate otherwise. Postoperative studies
Basic Echo pre discharge * * 2-3 weeks * *
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6 months * * 18 months * * Approx. every 2-3 yrs *
Individualize ECHO at 18-month visit depending for example on degree of AV valve regurgitation or evidence of subaortic stenosis.
15.9 Coarctation of the Aorta
Preoperative studies Primary isolated coarctation: All require Echo. In older patients where echo images are less clear CMR or CT angiography may be required to show clearly the anatomy of the aortic arch. For those < 6 months surgical repair unless very poor ventricular function or coexisting infection. For those between 6 months and 8 years individualise to either surgery or balloon angioplasty. Above 8 years stent implantation is becoming treatment of choice unless contraindicated by anatomy. Coarctation with associated critical aortic valve stenosis: Outside spectrum of hypoplastic left heart syndrome treatment depends on severity of each lesion. Coarctation with associated mitral valve disease: Outside spectrum of hypoplastic left heart syndrome, balloon dilatation or surgery of coarctation if > 6 months (as above), then reassess mitral valve by Echo. Patients with coarctation and complex intracardiac anatomy: may require catheterization or CT or CMR before surgery. Coarctation / VSD: a) Large VSD on echo: repair coarctation + primary VSD repair (or pulmonary artery banding) b) Small VSD: repair coarctation, no PA band c) Multiple muscular VSDs or apical VSD on echo: repair coarctation with PA band. Will require catheter before complete repair.
Postintervention Always obtain a right arm blood pressure and palpate the femoral pulses. In small infants a lower limb blood pressure should also be performed.
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Basic Echo CMR CT pre discharge * * 6-8 weeks after 2 weeks * * balloon angioplasty or 3 months * * stenting 6 months * * 12 months * individualize subsequent every * individualize + [see 2 years comments below]
Recoarctation All require echocardiogram, prior to intervention. In older patients where echo images are less clear CMR or CT angiography may be required to show clearly the anatomy of the aortic arch. Difficulty in palpating femoral pulses or a cuff blood pressure gradient of > 20mmHg indicates the need for re-intervention. Older children with both native and recurrent coarctation should be considered for stent implantation (cut-off around 30 kg) unless morphology of lesion very discrete. Post intervention all require MRI in adolescence. The remainder of follow-up is similar to post surgical repair.
15.10 Coronary artery fistulas
Preoperative investigations All require echo, followed by cardiac catheterization. CT angiography can also help delineate anatomy. The timing for cardiac catheterization will be dictated by symptoms, age and significance of fistulas (those with dilated main coronaries). Either catheter intervention or surgical ligation may be considered depending on the anatomy of the fistulas. Small fistulas: As defined by those without main coronary artery enlargement. These may be followed conservatively as many will regress spontaneously with time. Moderate to large fistulas: These will invariably need treatment either by catheter or surgery, depending upon anatomy.
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Postintervention investigations
Basic Echo Myocardial Holter perfusion pre discharge * * 3 months * * 12 months * * every 1-2 yrs * * individualize consider
Anticoagulation may be considered for some cases with residual large redundant proximal coronaries until spontaneous regression occurs.
15.11 Corrected Transposition (A-V and V-A discordance)
Preoperative studies All require echocardiography & may require cardiac catheterization prior to complete surgical repair if VSD +/- PS. Holter monitor suggested prior to repair. Surgical management dictated by lesion
CTGA
sig (L) AV reg sig( L) AV VSD VSD
no VSD + VSD +PS no PS
Options: a) cTGA+VSD+PS/PAtresia. Atrial switch plus Rastelli (connect morphologic LV to aorta through VSD + morphologic RV-PA conduit and an atrial switch [Mustard or Senning]). May require initial shunt. b) cTGA+VSD+noPS: Initial banding and then consider double switch. c) In some cases, it is appropriate to connect LV to PA if systemic AVV competent (physiological repair). d) Pulmonary artery banding if significant LAVV regurgitation with TOE mediated adjustment of band in theatre to optimise regurgitation and LV function. e) No intervention if patient is well balanced (e.g. VSD & PS) with no significant AVV regurgitation
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Postoperative studies individualize according to surgical intervention.
Basic Echo Holter Exercise pre discharge * * 2 weeks * * 6 months * * 18 months * * * subsequent * * ? if > 6 years every 1-2 yrs
15.12 Double Outlet Right Ventricle
Preoperative studies All require echo study and may require cardiac catheterization +/- TOE prior to complete surgical repair. Initial palliation does not need catheterization.
DORV
Remote D/committed Subpulmonary Subaortic
VSD VSD VSD VSD
With significant PS initial palliative shunt with later repair. With significant additional muscular VSDs: initial PA band and later full repair With aortic arch obstruction: initial repair with PA band and later full repair. If initial palliation with subpulmonary VSD, may also require early BAS. Postoperative studies
Basic Echo MRI & MVO2 pre discharge * * 2-3 weeks * * 6 months * * 18 months * * subsequent follow-up * individualize adolescent
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15.13 Fontan (Total cavopulmonary Connection (TCPC) pathway for complex lesions
Complex lesions Definition: This encompasses all cases headed for a TCPC (univentricular A-V connections, and HLHS lesions with significant ventricular hypoplasia, straddling AV valves, remote VSD.... etc) Preoperative studies All require echo study prior to surgical intervention. First stage Norwood type procedures (see later), PA bands and BT shunts may be performed without prior cardiac catheterization although balloon atrial septostomy may be necessary if the ASD is restrictive, particularly with small or absent LAVV. Postoperative studies Cases of HLHS will remain as in patients until after the Glenn procedure. Other conditions will be discharged after 1st procedure with close monitoring in clinic and at home. Community nurses will check saturations with frequency at consultant discretion. Prior to SECOND procedure (bidirectional superior Cavo pulmonary anastomosis) a catheter is required. In some cases, with the branch pulmonary arteries clearly seen on imaging and Doppler assessment indicates a low pulmonary artery pressure it might be possible to proceed without catheter. Consider bidirectional Cavo pulmonary shunt at 3-6 months of age. Additional procedures for subaortic stenosis, unobstructed TAPVC or AVV regurgitation best timed with this procedure.
Basic Echo Oximetry
pre discharge * * * (& Hb)
3 months * * *
6 months * individualize *
18 months * * *
Prior to the THIRD procedure (completion of TCPC) between 2 and 3 years all require catheterization or MRI with SVC pressure measurement. During catheter attention is paid to a) PA pressure & size; b) A-V valve function; c) subaortic stenosis; and d) ventricular function + mass. Based on these findings a decision of either high or low risk TCPC is made, as well as whether fenestration recommended.
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Post TCPC Include oximetry with basic investigations
Basic Echo Holter Cath CMR pre discharge * * 2-3 weeks * * 6 months * To close any persistent fenestration 18 months * *
1-2 years * * * Older children 2-3 yearly
Anticoagulation as per coagulation guidelines. Renal and hepatic function should be checked every 2 years. All cases of HLHS will have their data reviewed intermittently in the HLHS specialist JCC MDT meeting. Timing decided by lead cardiologist and surgeon for the patient.
15.14 Hemitruncus arteriosus
Preoperative studies All require Echo at time of initial presentation. Cardiac catheterization reserved for case where concerns about pulmonary vascular resistance or if part of a complex lesion. Postoperative studies
Basic Echo Lung perfusion pre discharge * * 2-3 weeks * * 6 months * * 18 months * * Every 2 years * individualize As indicated
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15.15 Hypertrophic Cardiomyopathy
Basic evaluation Cardiomyopathy screen (see appendix 1) to try and evaluate cause. Screening of family, involving primary and secondary care teams. If patient has significant obstruction, pharmacologic manipulation attempted (propranolol and disopyramide). If medical therapy fails and patient symptomatic, consider surgical myectomy. After initial assessment, if no surgery yearly follow-up with basic, Holter & echocardiogram. If surgery performed follow-up as per sub-aortic stenosis.
15.16 Hypoplastic Left Heart Syndrome
All require detailed Echo study prior to surgery, to confirm diagnosis and exclude significant associated abnormalities. All patients transferred to the paediatric intensive care unit prior to surgery for pre-op management as HLH pathway. Important to assess ASD size and pressure gradient, degree of A-V valve regurgitation, associated pulmonary venous abnormalities and size of ascending aorta. Patients counselled: Active treatment is Norwood procedure. Postoperative studies (Norwood)
Basic incl. Echo Cath oximetry pre discharge * * monthly intervals * * 3 months * * *
The post Norwood echo should include an assessment of RV pressure, which is then compared to leg blood pressure, as arm BP readings may be misleading, resulting in missed diagnosis of arch obstruction. Home monitoring required interstage I and II as per liaison nurse protocol. After the 3-month catheter the patient should follow the pre and post Bidirectional Cavopulmonary shunt/Fontan track. [for example, a bidirectional cavopulmonary shunt at about 3-4 months and TCPC at around 24 months]
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15.17 Interrupted Aortic Arch
Preoperative studies All require Echo prior to complete repair, cardiac catheterization, CMR or CT angiography if echo assessment unclear. Postoperative studies Always obtain a right arm blood pressure and palpate the femoral pulses. In small infants a lower limb blood pressure should also be performed.
Basic Echo Holter pre discharge * * 2-3 weeks * * 3 months * * 6 months * * 1 year * * *if previous concerns of arrythmia every 1-2 years * individualize
MRI or CT of arch if obstruction is suspected at follow up. Baseline Holter, MRI and exercise testing at 10 years, 14years and transfer to ACHD care. Consider CT if child unable to comply with MRI. GA MRI can be considered. Screening cholesterol at 10 years of age.
15.18 Kawasaki Disease
Normal Coronaries (-2 Basic (include ECG!) Echo at presentation * * 1-2 weeks * * 4-6 weeks * * 6 months * * Issued: April 2019 Version: 001 Page | 62 Link - Contents Page ▪ No aspirin beyond the initial 6 to 8 weeks ▪ After 12 months for follow up at discretion of cardiologist. ▪ If persistently normal coronaries discharge at 12 months ANYONE WHOSE CORONARIES ARE ABNORMAL at any stage are for referral to the specialist KAWASAKI clinic (led by Prof Fraisse, Prof Di Salvo and ID Kawasaki experts from ST Mary’s Hospitals including Jethro Herberg). Below is a guideline but please note care is individualized in the Kawasaki clinic and at discretion of experts leading the clinic. Ectasia / Dilatation (2< Z<2.5) Basic (include ECG!) Echo at presentation * * 1-2weeks * * 6 weeks * * 6 months * * yearly if persists * * ▪ Low dose aspirin (3-5mg/kg/day) until coronaries in normal range ▪ Discharge when coronaries are within normal range Aneurysms (Z>2.5) Echo at least 2/per week until the luminal dimensions stop progressing. And 1/week in the first 45 days in the present of expanding large or giant aneurysm to detect coronary thrombosis. Then monthly until the 3rd month. See AHA guidelines for further details. 1) Small coronary artery aneurysm (Z = 2.5 – 5.0) Current or persistent Regressed to normal or dilatation only ▪ Assess 6months and then annually ▪ Consider CT and MRI every 2-5years ▪ Assessment for inducible myocardial ischemia with Stress Echo/ MRI every 1-3 years ▪ Long term antiplatelet therapy, until the aneurysms regress ▪ No restriction to physical activity 2) Medium coronary artery aneurysm (Z = 5.0 – 10.0) Current or persistent Issued: April 2019 Version: 001 Page | 63 Link - Contents Page Regressed to small aneurysms ▪ Assess at 3 months, 6months and 12 months, then annually ▪ Coronary CT can be considered and then reconsider CT or MRI every 1-3 years ▪ Assessment for inducible myocardial ischemia with Stress Echo every 1-3 years ▪ Long term antiplatelet therapy ▪ Consult with Anticoagulation service, as increased anticoagulation may be considered (Warfarin/LMWH) ▪ No contact sports. No other restriction to physical activity. ▪ Females: reproductive counselling (warfarin risk) 3) Giant coronary aneurysms (Z >10) Multiple complex aneurysms without obstruction, current or persistent Regressed to medium aneurysm Regressed to small aneurysm Regressed to normal or dilatation ▪ Assess at 3moths, 6months, 9months and 12 months, then every 6 months ▪ Consider CT at 6months and then reconsider CT or MRI every 1-3 years ▪ Assessment for inducible myocardial ischemia yearly with non-invasive stress testing (stress echo or exercise test): Coronary CT-angiogram if myocardial ischemia demonstrated by stress test. ▪ Cardiac catheterization including coronary CT angiogram 6 -12 months in case of suspected or confirmed myocardial ischemia (ECG, imaging) ▪ Long term antiplatelet therapy ▪ Consult with Anticoagulation service, as increased anticoagulation may be considered (warfarin/LMWH) ▪ No contact sports. No other restrictions of physical activity. ▪ Females: reproductive counselling (warfarin risk) ▪ CMC (Coordinate My Care, with directives to the ambulance) +ESP (Emergency Care Plan) +SCP (School Care Plan) if age appropriate. 4) Patients with coronary artery obstructions (calcifications/clots) ▪ Long term antiplatelet therapy ▪ Warfarin, target INR 2 – 3.5 ▪ Restricted physical activity as guided by stress test. No contact sports ▪ KD clinic every 6monyhs ▪ Myocardial perfusion imaging including stress test (patient >10 years) annually ▪ Coronary CT if myocardial ischemia demonstrated by stress test. ▪ For acute chest pain, follow ACS- KD guidelines ▪ CMC+ECP+SCP (if age appropriate) 15.19 Patent Arterial Duct Preoperative studies Issued: April 2019 Version: 001 Page | 64 Link - Contents Page With typical findings verified by Echo, occlusion by catheter is first line management at Royal Brompton. Under rare circumstances surgery may be required. If not in failure then manage medically until 5 Kg, for device occlusion. Tiny silent ducts do not require treatment. Postop/occlusion studies Basic Echo Occlusion 3/12 3/12 Surgery 3/12 3/12 Discharge surgical patients after one follow-up visit. Discharge device patients after 2 years. If a residual leak present review yearly. If clinically silent patent duct detected and patient over 2 year of age, discharge to PEC/GP to listen in 2-3 years and refer if murmur develops or alternatively offer follow up in 5 years. 15.20 Pulmonary atresia and ventricular septal defect Preoperative studies If no aortopulmonary collaterals may not need a catheter if anatomy is clear. If aortopulmonary collaterals are suspected, all need a catheter or CT prior to surgery. NB blood products and conotruncal lesions (see above). Pulmonary Arteries (confluent + PDA) <3.0 mm PA's >3.0 mms PA's Outflow tract patch BT shunt, later repair ideally when adequate conduit can be accepted Pulmonary Arteries (non confluent, blood supply varied) Individualize, RV outflow tract patch and staged unifocalization with occlusion collaterals causing dual supply with eventual RV-PA conduit ± VSD closure. RV-PA conduits will need subsequent replacement, timing depending upon the amount of conduit stenosis, regurgitation and right ventricular size and function. Postoperative studies (Repaired) Issued: April 2019 Version: 001 Page | 65 Link - Contents Page Basic Echo Cath CMR& MVO2 pre * * discharge 2-3 weeks * * 6 months * * 12 months * * 18 months * * 1-2 yearly * * If concern In adolescence Investigations may be varied as to whether, angioplasty, stenting or embolization are necessary. 15.21 Pulmonary Valve Stenosis Preoperative All require Echo prior to intervention. If classic dysplastic valve take care to exclude associated pathology on the left side, e.g. Noonan’s syndrome. If the peak instantaneous gradient is greater than 60mmHg, then a balloon dilatation should be attempted. For those with gradients that do not require intervention, 6 monthly follow-up with echo and ECG for those < 2 years of age, 1-2 yearly > 2years. Consider 3yrly appointments and then discharge from follow-up of adolescents whose Doppler gradient is less than 25 mmHg. Patients with a dysplastic valve should have an attempted dilatation, however surgery is usually necessary. In the first week of life balloon angioplasty should be performed on Prostaglandins, even if the right ventricle is small. Postintervention Basic Echo CMR 3 months * * 1-2 yearly * * If significant pulmonary regurgitation Issued: April 2019 Version: 001 Page | 66 Link - Contents Page 15.22 Pulmonary Atresia with Intact Ventricular Septum Preoperative studies All patients require Echo prior to intervention. Cardiac catheterization may be indicated if there is suspicion of a RV dependent coronary circulation. BAS Initial Rx Goal Right ventricle no RF perforation of PV Biventricular repair (infundibulum patent, [+/- BT shunt] not RV dependent circulation) Bidirectional CP shunt/TCPC or 1 1/2 ventricle repair Right ventricle (atretic infundibulum or RV dependent circulation) yes BT shunt TCPC ± atrial septostomy before shunt Postoperative studies For those on a Fontan track, see pre Fontan assessment. For those for a biventricular repair, without a BT shunt, follow as per isolated pulmonary valve stenosis. For those with a borderline RV size and a BT shunt, Echo and catheterization prior to deciding if shunt and/or ASD can be closed surgically or by catheter. 15.23 Secundum Atrial Septal Defect Preoperative studies Only Echo with classical features, yearly review, with repeat echo prior to closure. Patients with a PFO (atrial communication < 3 mm ) and no right heart volume overload should be discharged. Issued: April 2019 Version: 001 Page | 67 Link - Contents Page In cases where the ASD size or location is uncertain a transesophageal echocardiographic study might be required prior to intervention. If the pulmonary veins can not be identified, a cardiac catheter/ CMR or CT angiogram may be required to show where the pulmonary veins connect. Postoperative studies Basic Echo Holter pre-discharge * * 2-3 weeks * * 6 months * 18 months * * every 5 years * * * for Sinus venosus For device closure first follow-up at 3 months and then 1-2 yearly, should remain on low dose aspirin until first follow-up visit. Discharge patients with secundum ASDs 5 years after treatment. Sinus venosus ASDs require continuing follow-up 2-3 yrly. 15.24 Fibromuscular Subaortic Stenosis Preoperative studies All require Echo assessment prior to surgical repair. If the mean gradient is 40-50 mmHg, then repair, especially in the presence of aortic regurgitation and left ventricular hypertrophy All require preoperative serial Echo assessment yearly. If there is an aortic commissural abnormality and/or aortic regurgitation, then earlier repair may be necessary. Postoperative studies Note that this lesion can recur Basic Echo pre-discharge * * 2-3 weeks * * 6 months * * 18 months * * every 2 to 3 years * * Issued: April 2019 Version: 001 Page | 68 Link - Contents Page 15.25 Tetralogy of Fallot Preoperative studies All require Echo prior to intervention. If undergoing a shunt procedure or repair then catheterization is unnecessary if the anatomy is clear. If there is concern of aortopulmonary collateral arteries, branch pulmonary artery stenosis or anomalous LAD across the right ventricular outflow tract, then further imaging (cardiac catheterization or CT angiography) is required. At catheterisation, “down the barrel” view for coronary arteries. Require catheter or CT angiography prior to a repair if previous shunt has been performed. NB blood products and conotruncal lesions (see above). Indications for repair Elective repair: between 3-9 months irrespective of whether or not have had a shunt. If symptomatic 3- 6 months repair unless small pulmonary arteries or severe spelling. Postoperative studies (repair) Basic Echo Holter MRI MVO2 pre- * * discharge 2-3 weeks * * 6 months * * 18 months * * every 2-3 * individualise * > 10 years at > 10 yrs yrs Catheter/CMR during follow-up if suspicion branch pulmonary artery stenosis or prior to pulmonary valve replacement. Most patients will require pulmonary valve replacement in adolescence/ early adulthood. Issued: April 2019 Version: 001 Page | 69 Link - Contents Page 15.26 Complete transposition of the great arteries (intact ventricular septum) Preoperative studies All patients require an Echo. If intracardiac anatomy and coronary pattern is clear, then balloon atrial septostomy can be performed if required in the PICU or NICU. If there is coarctation, leave on prostaglandin prior to repair of both lesions. Infants beyond 6 weeks of age may require 2-stage repair to prepare LV before switch. Postoperative studies Basic Echo Catheter & Holter CMR pre discharge * * 2-3 weeks * 6 months * * * 6-12 months if previous arrythmia 18 months * * every 2 years * * Individualise • Age 4yrs, 10 & 14yrs and at transfer to ACHD team Consider Lipid panel age 10 yrs. Catheter or CMR to look for branch PS or if there are concerns on echo. Exercise test and CMR myocardial perfusion imaging at 14 years of age, sooner if concern of myocardial ischaemia 15.27 Truncus Arteriosus Preoperative studies All patients require an ECG & Echo prior to surgical repair. Cardiac catheterization reserved for cases where anatomy unclear or there is concern of the pulmonary vascular resistance. Genetic testing for 22q11 deletion should be performed. If no result available presume diagnosis and use irradiated blood products and conotruncal lesions. Early repair is recommended unless there are complicating lesions. Issued: April 2019 Version: 001 Page | 70 Link - Contents Page Postoperative studies Basic Echo MVO2 & CMR Holter pre discharge * * 2-3 weeks * * 6 months * * If previous arrhythmia concerns .to be done at 1 yr of age* 18 months * * every 2-3 years * * > 10 years age If previous arrhythmia concerns .to be done at 4 yr of age* Repeat Holter at 10 & 14 yrs of age. Cardiac catheter or CT/MRI if concern regarding branch pulmonary arteries on echo. Perfusion scan may be required if echo suggests asymmetric pulmonary blood flow. The RV-PA conduit will need replacement or intervention if significant stenosis/ regurgitation Holter before conduit replacement. Screening cholesterol aged 10 yrs. Holter, Exercise test, MRI to be considered at 10 & 14 years of age and at transfer to ACHD care. 15.28 Ventricular Septal Defect Preoperative studies Patients who require surgery need a detailed Echo prior to surgery. Associated lesions such as RV muscle bundles, subaortic stenosis and aortic valve prolapse should be excluded or confirmed. Catheterization if concern regarding pulmonary resistance or if associated lesions are not adequately imaged. Those with significant multiple muscular VSDs should have a PA band. Those with a perimembranous VSD and small associated muscular VSDs should be repaired and may or may Issued: April 2019 Version: 001 Page | 71 Link - Contents Page not require a pre-op catheterization, depending on the size and location of the associated muscular defects. VSD does not always require intervention, follow-up individualized. For those with perimembranous VSDs repeat echo recommended 2-3 yearly to exclude the development of associated lesions. Patients with a small restrictive muscular VSD with no evidence of significant left heart dilatation can be discharged back to Paediatrician or GP age 6-12 months of age with advice to re-refer after 5 years if murmur still present. Postoperative studies Basic Echo pre discharge * * 2-3 weeks * * 6 months * 18 months * + 5 years * * Aortic regurgitation is an indication for surgical closure. If mild, child should be follow-ed up yearly to look for progression. Discharge at 5 years post surgery if no concerns and normal ECG. Patients with VSD device closure should not be discharged currently, but followed-up every 1-2 2 years 15.29 Authors Written by AG Magee June 2009. 1st Revision by N Naqvi, Michael Rigby & Rodney Franklin February 2011, 2nd Revision N Naqvi – circulated and checked by cardiology consultant group May 2019. 15.30 References 1. Wernovsky G, Lihn SL, Olen MM. Creating a lesion-specific "roadmap" for ambulatory care following surgery for complex congenital cardiac disease. Cardiol Young. 2017;27(4):648-62. 2. Hass NA, Schirmer KR et al. Guidelines for the Management Of Congenital Heart Diseases In Childhood and Adolescence. Cardiol Young. 2017;27(Suppl 3) S1-105. Issued: April 2019 Version: 001 Page | 72 Link - Contents Page 16 Appendix 3 – Management Guidelines for Adults with Congenital Heart Disease RBH has adapted American Heart Association Guidance for management of Adult CHD patients. 16.1 Physiological States A - NYHA FC I symptoms - No hemodynamic or anatomic sequelae - No arrhythmias - Normal exercise capacity - Normal renal/hepatic/pulmonary function B - NYHA FC II symptoms - Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular - enlargement, mild ventricular dysfunction) - Mild valvular disease - Trivial or small shunt (not hemodynamically significant) - Arrhythmia not requiring treatment - Abnormal objective cardiac limitation to exercise C - NYHA FC III symptoms - Significant (moderate or greater) valvular disease; moderate or greater - ventricular dysfunction (systemic, pulmonic, or both) - Moderate aortic enlargement - Venous or arterial stenosis - Mild or moderate hypoxemia/cyanosis - Hemodynamically significant shunt - Arrhythmias controlled with treatment - Pulmonary hypertension (less than severe) - End-organ dysfunction responsive to therapy D - NYHA FC IV symptoms - Severe aortic enlargement - Arrhythmias refractory to treatment - Severe hypoxemia (almost always associated with cyanosis) - Severe pulmonary hypertension - Eisenmenger syndrome - Refractory end-organ dysfunction Issued: April 2019 Version: 001 Page | 73 Link - Contents Page 16.2 Atrial Septal Defect • Referral to Level 1 centre • Type of ASD and size confirmed via TTE ECHO Confirm Diagnosis • Chest X-ray; Blood test, ECG; CMR; MVO2; • TOE ECHO might be necessary to assess type and size of ASD and asscoaited defects (venous drainage) • Cardiac Catheter may be required to assess for pulmonary vacsular resistence, Assessment LV diastolic dysfunction and any other associated defects. • Device closure is first choice intervention for ASD OS if size and rims are adequate. • Antiplatelet therapy recommended for at least 6 months post percutaneous intervention. Intervention • Surgical intervention is recommended for : ASD OS not suitable for percutaneous closure; primum ASD; and most sinous venous ASDs. • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks to assess the device position, pericardial effusion; residual shunt; TR; PAP. • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor Post intervention should be ordered Follow-up • Patients with significant residual shunts; elevated PAP; or arrhythmias before or after repair should be reviewed at a level 1 centre at least once every 1-2 years. • Patients with Eisenmenger syndrome or PH associated with closed defects Long-term should have specialist PHT follow-up every 3 - 12 months depending on PHT Follow-up therapies and severity (refer to PH4 clinic). Issued: April 2019 Version: 001 Page | 74 Link - Contents Page 16.3 Ventricular Septal Defect • Referral to Level 1 Centre Confirm • Type of VSD and size confirmed via TTE ECHO. Diagnosis • Chest X-ray; Bloods, ECG; CMR; MVO2; • RH Cardiac Catheter may be required to assess PVR, Qp/Qs and any other associated Assessmen t defects. • Closure should be considered: • If unrepaired VSD with significant shunt (Qp/Qs >1.5) and PVR not elevated Interventio n • If restrictive VSD but progressive AR, LV dilatation or history of endocarditis • Surgical closure remains the treatment of choice in most cases. • Device closure can be considered when the defect is accessible for percutaneous intervention or in patients with increased risk factors for surgery. • Post procedure CNS Review with ECHO within 6-8 weeks to assess for pericardial effusion, residual shunt, TR and estimated PAPs. Post Interventio • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be n Follow-up ordered. • Patients with LV dysfunction; residual shunt; AR; RVOTO; LVOTO should be seen every 1 year at Level 1 centre • Patients with small VSD (native or residual) and normal LV; normal PAP; Long-term asymptomatic; and no other lesion should be reviewed every 3 -5 years. Follow-up • After device closure annual follow-up for the first 2 years then if no complications follow-up should be every 2-4 years • After surgical closure without residual abnormalities follow-up should be every 3-5 years. • Patients with Eisenmenger syndrome or PH associated with closed defects should have specialist PHT follow-up every 3 - 12 months depending on PHT therapies and severity (refer to PH4 clinic). • If residual shunt after closure OR Eisenemenger Syndrome --> Endocarditis Prophylaxis for invasive dental procedures is recommended Issued: April 2019 Version: 001 Page | 75 Link - Contents Page 16.4 Atrioventricular Septal Defect •Referral to Level 1 Centre • Diagnosis confirmed via TTE ECHO: • - Complete AVSD is characterized by the presence of a common AV annulus guarded by 5 leaflets; the septal defect is in the crux of the heart, extending to both the intral atrial and intraventircular septum. •- Partial AVSD or Primum ASD - the anterior and posterior bridging leaflets are fused centrally Confirm Diagnosis creating seperate left and right-sided orifices; the septal defect is only at atrial level. •- Transional or intermediate AVSD has an incomplete atrial and VSDs and/or incomplete abnormalities of the common atrioventricular valve •Chest X-ray; ECG; Bloods, CMR; MVO2; •RH Cardiac Catheter may be required to assess for PHT and any other associated defects such Assessment as Eisenmenger syndrome. •Primary surgical repair is recommended if left-to-right shunt (Qp:Qs ≥1.5:1), PA systolic pressure less than 50% systemic and pulmonary vascular resistance less than 1/3 systemic. •If Eisenmenger Syndrome Physiology present then intervention is contraindicated. •Operation for discrete LVOT obstruction in adults with atrioventricular septal defect is reasonable with a maximum gradient ≥50 mm Hg a lesser gradient if HF symptoms are Intervention present, or if concomitant moderate-to-severe mitral or AR are present. •Severe LAVV stenosis or regurgitation should bve followed as per the VHD guidelines •Post proedure ECHO within 6-8 weeks to assess for pericardial effusion; residual shunt; TR; PAP. Post Intervention Follow-up •ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be ordered. •Patients with residual shunt; AV malfunction; LV & RV enlargement and dysfunction; PAP elevation; LVOTO; and arrhythmias/conduction disease hould have follow-up every 1-2 years depending on the severity of the abnormality. •Patients with no residual abnormalities and no AV valve malformation should be reviewed every 2-3 years. Long-term Follow- up •Holter monitor should be considered as complete heart block can appear as late as 15 years after surgery. • Patients with Eisenmenger syndrome should have specialist PHT follow-up every 3 - 12 months depending on PHT therapies and severity. Issued: April 2019 Version: 001 Page | 76 Link - Contents Page 16.5 Patent Ductus Arteriosus • Referral to Level 1 Centre • Diagnosis confirmed via ECHO showing size of the defect, direction of shunt and Confirm Diagnosis LV volume overload. • Chest X-ray; ECG; Bloods. CMR; MVO2; Cardiac Catheter may be required to assess PVR • Small and Moderate size PDA (but not tiny) usually cause significant left-to-right shunt, LV overload and increased pulmonary pressures. Assessment • Unrepaired large PDA patients will likely have developed Eisenmenger Syndrome when they arrive to adulthood. • Device closure is the intervention of choice • Surgery is reserved for patients where the duct is too large for device closure or with unsuitable anatomy with aneurysm formation. Intervention • If Eisenmenger Syndrome present then intervention is contraindicated • Post procedure CNS Review with ECHO within 6-8 weeks to assess device position, LV size and function; PAP; residual shunt; and associated lesions. Post Intervention • Endocardiis prophylaxis on the first 6 months after closure. Follow-up • Patients with no residual shunt, normal LV and normal PAP should have follow- up 6 -12 months after intervention with an echocardiogram. • If surgical closure and no abnormality found, patients can be discharged from specialist follow-up. If device closure, patients should be seen every 3-5 years. • Patients with LV dysfunction or any other associated abnormalities should have Long-term Follow- follow-up every 1-3 years depending on the severity. up • Patients with Eisenmenger syndrome or PH associated with closed defect should have specialist PHT follow-up every 3 - 12 months depending on PHT therapies and severity. Issued: April 2019 Version: 001 Page | 77 Link - Contents Page 16.6 Congenital Valvular Aortic Stenosis • Referral to Level 1 Centre Confirm Diagnosis •Chest X-ray; ECG; Bloods, CMR; MVO2 (except if symptomatic AS); •Patients with bicuspid aortic valve should be screened for Aorthopathy and Aortic Coartation. Assessment •Stress ECHO may be required to assess severity of AS and associated AR •Balloon valvuloplasty may be considered only in selected adolescents and young adults with non-calcified valves and no more than mild AR. •Valve Replacement is the treatment of choice in patients with calcified valves - with mechanical Intervention or biological valves. •Aortic root or ascending aorta replacement should be cnsidered when are > 45mm •The Ross Procedure can be considered in those patients of child-bearing age where anticoagulation would be avoided. •Transcatheter valve implantation should only be considered in those unsuitable for conventional surgery. •Post procedure CNS review with ECHO within 6-8 weeks to assess the AVR, residual AR, root/ascending aorta, LV function; LVH and associated lesions. Post Intervention •ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be ordered. Follow-up •Bloods to check Hb, Iron studies and inflammatory parameters. • At risk Stage A - with Vmax <2m/s - OPA with ACHD Team with ECG and ECHO every 36-60 months (Exercise test as required) • Progressive Stage B - OPA with ACHD Team with ECG and ECHO- mild severity Vmax 2.0-2.9 m/s - every 36-60 months; moderate severity Vmax 3.0-3.9m/s - every 12-24 months), (Exercise test as required). Long-term Follow- up • Severe Stage C - OPA with ACHD Team with ECG and ECHO every 6-12 months (Exercise test as required). •Aortic dilation >4.5cm OPA with ACHD Team with ECG and ECHO every 12 months (Exercise test, CMR or CT as required). •It is reasonable to screen first degree relatives of patients with bicuspid aortic or unicuspid aortic valve with ECHO for valve disease and aortopathy. Issued: April 2019 Version: 001 Page | 78 Link - Contents Page 16.7 Subaortic Stenosis • Referral to Level 1 Centre • SubAS may be first diagnosed in adulthood and high level of suspicion is required, as it may be confused with hypertrophic obstructive cardiomyopathy when LV hypertrophy of Confirm Diagnosis sufficient severity has developed such that subAo membrane is less evident on imaging. • Chest X-ray; ECG; Echo, Bloods, CMR; • Special attention needs to be given to the degree of AR • Cardiac Catheterization may be required to assess LVOT gradient and any other associated defects. If ischemic symptoms, coronary artery disease need to be rule out. Assessment • Stress testing for adults with LVOT obstruction to determine exercise capacity, symptoms, ECG changes, or arrhythmias may be reasonable in the presence of otherwise equivocal indications for intervention. • Surgical intervention is recommended for: • Adults with subAS with a peak gradient ≥50mmHg and HF or ischaemic symptoms, and/or LV systolic dysfunction attributable to subAS. • Peak gradient <50mmHg and HF or ischaemic symptoms, and/or LV systolic Intervention attributable to subAS. • Surgery can be considered in asymptomatic adults with subAS and at least mild AR and peak gradient ≥50mmHg to prevent progression of AR • Post procedure CNS review with ECHO within 6-8 weeks to assess residual AR, residual LVOT, LV function; LVH and associated lesions. • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be Post Intervention ordered. Follow-up • Bloods to check Hb, Iron studies and inflammatory parameters. • Physiological Stage A - OPA with ACHD Team with ECG and ECHO every 24 months (Exercise test as required) • Physiological Stage B - OPA with ACHD Team with ECG and ECHO every 24 months (Exercise test as required) Long-term Follow- up • Physiological Stage C - OPA with ACHD Team every 6-12 months with ECG and ECHO every 12 months. Exercise test every 24 months. • Physiological Stage D - OPA with ACHD Team every 3-6 months with ECG and ECHO every 12 months. Exercise test every 12 months. Issued: April 2019 Version: 001 Page | 79 Link - Contents Page 16.8 Supravalvular Aortic Stenosis • Referral to Level 1 centre • Supravalvular aortic stenosis is a relatively rare condition overall but is seen commonly in patients with Williams syndrome or homozygous familial hypercholesterolemia. Confirm Diagnosis • The stenotic ridge tends to occur distal to the coronary artery orifices at the sinotubular junction. • Patients with supravalvular AS have higher risk of CAD and SCD. • ECG, bloods, Chest Xray, ECHO. • Aortic imaging with with TOE, CMR, CT angiography is recommended in adults with Assessment Williams syndrome or patients suspected of having supravalvular AS. • Coronary imaging is recommended in patients with Williams syndrome and supravalvular AS presenting with symptoms of coronary ischaemia • Surgical repair is recommended for adults with supravalvular AS (discrete or diffuse) and symptoms or decreased LV systolic function deemed secondary to aortic obstruction. Intervention • Coronary artery revascularisation is recommended in symptomatic aduts with supravalvular AS and coronary ostial stenosis. • Post procedure CNS Review with ECHO within 6-8 weeks. • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be ordered Post intervention Follow-up • Bloods to assess Hb, iron studies and inflammatory parameters. • Physiological Stage A - OPA with ACHD Team with ECG and ECHO every 24 months, CMR every 36-60 months (Exercise Test as required). • Physiological Stage B - OPA with ACHD Team with ECG and ECHO every 24 months, CMR every 36-60 months, Exercise test every 24 months. Long-term Follow- • Physiological Stage C - OPA with ACHD Team every 6-12 months with ECG and ECHO up every 12 months. CMR every 36-60 months, Exercise test every 24 months. • Physiological Stage D - OPA with ACHD Team every 3-6 months with ECG and ECHO every 12 months, CMR every 36-60 months, Exercise test every 12 months. Issued: April 2019 Version: 001 Page | 80 Link - Contents Page 16.9 Coarctation of the Aorta • Referral to Level 1 Centre Confirm • Diagnosis confirmed via ECHO Diagnosis • Chest X-ray; Bloods,ECG; CMR; MVO2; Cardiac Catheter may be required to assess any other associated defects. • Physical examination should include BP measured in upper and lower limbs Assessment • If hypertension present then 24hr BP monitor should be arranged. • Stenting is the treatment of choice in adults in native CoA with appropriate anatomy • For adults with recurring or residual CoA angioplasty with or without stent implantation can be effective. • Surgical treatment in adults where necessary can be complicated and ascending to Intervention descending conduits may be preferable in cases of difficult anatomy. There is a risk of spinal cord injury with surgical repair of CoA. • Post procedure CNS Review with Bloods + ECHO within 6-8 weeks to assess for stent position and residual CoA. Post Intervention • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be Follow-up ordered. • All patients with CoA require regular follow up every 1-2 years. BP on upper and lower limbs should be assessed every visit. • Imaging of Aorta with CMR (ot CT if CMR not feasible) should occur every 3-5 years to document the post op or post intervention anatomy and complications such as restenosis, stent fracture or migration, or aneurysm formation. Long-term • Imaging may be required more frequently in known bicuspid aortic valve physiology Follow-up or Shone syndrome. • BP should be assessed at rest, ambulatory, or exercise test. If HTN, treatment should be start inmediatly. Issued: April 2019 Version: 001 Page | 81 Link - Contents Page 16.10 Isolated Pulmonary Regurgitation after repair of Pulmonary Stenosis • Referral to Level 1 Centre • TTE ECHO to assess PR severity and RV size and function Confirm Diagnosis • Chest X-ray; Bloods, ECG; CMR; MVO2 Assessment • In symptomatic patients with moderate to severe PR and RV dilatation/dysfunction -- > Pulmonary Valve Replacement (PVR) is recommended. Intervention • In asymptomatic patients with moderate to severe PR with progressive RV dilation and/or RV dysfunction, PVR may be reasonable. • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks to assess PVR, RVOT; RV function; and associated lesions. Post Intervention • ECG to ensure sinus rhythm. I f concerned about arrhythmia holter monitor should Follow-up be ordered. • Before intervention: • Moderate or greater PR and RV enlargement without symptoms and no evidence of progressive RV dilation and/or RV dysfunction and/or progressive decrease in exercise capacity - OPA with ACHD Team with ECG and ECHO every 12-24 months. (CMR and Exercise test as required). Long-term • After intervention: Follow-up • PVR and mild PR - OPA with ACHD Team with ECG and ECHO every 24 months; (Exercise test as required) • PVR and significant PR or PS - follow up with ECHO every 12months; (MRI/Exercise test as required) • Endocarditis prophylaxis is recommended after PVR Issued: April 2019 Version: 001 Page | 82 Link - Contents Page 16.11 Right Ventricular Outflow Tract Obstruction • Referral to Level 1 Centre • RVOTO can occur at the subinfundibular; infundibular; valvular or supravalvular levels. Can be isolated or at multiple levels. Confirm Diagnosis • RVOTO level, severity and RV funcion should be confirmed via TTE ECHO. • ECHO; Chest X-ray; Bloods, ECG; CMR; MVO2; • Cardiac catheterisation may be required to assess severity of RVOTO • V/Q scan may be required to assess lung perfusion abnormalities. Assessment • Severity: • Mild RVOTO - gradient <36mmHg (Peak velocity <3m/s). • Mod RVOTO - gradient 36-64mmHg (Peak velocity 3-4m/s). • Severe RVOTO - gradient >64mmHg (Peak velocity >4m/s). • Balloon valvuloplasty is the first choice in clasic domed valvular PS and can even be considered in dysplastic type of valves. Intervention • Surgical valvulotomy or PVR might be necesarry in patients with significant infundibular stenosis; hypolplastic pulmonary annulus; dysplastic pulmonary valves or other associated lesions such as severe PR or severe TR. • Post procedure CNS Review with Bloods + ECHO within 6-8 weeks to assess PVR, residual PS or PS, RVOTO; RV dysfunction; Post Intervention • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be Follow-up ordered. • Prior to intervention: If Moderate to Severe RVOTO, follow-up should be every 6 - 18 months depending on the severity of RVOTO, rate of progression and RV function Long-term • Post intervention follow-up: every year for the first 2 years with ECHO and ECG. Follow-up • Patients with mild RVOTO without evidence of progression can be followed up every 3-5 years. • CMR and MVO2 should be considered every 3-5 years depending on ECHO findings. • Endocarditis prophylaxis is recommended after PVR Issued: April 2019 Version: 001 Page | 83 Link - Contents Page 16.12 Branch and Peripheral Pulmonary Stenosis • Referral to Level 1 Centre • TTE ECHO to confirm diagnosis and evaluate RV pressure and function Confirm Diagnosis • Bloods, ECG, MVO2 and Chest X Ray. • Cardiac MRI / Cardiac CT scan to visualise anatomic and branch PA anatomy. Assessment • Pulmonary Perfusion testing can be used to quantify relative pulmonary blood flow. • Balloon angioplasty or stenting of peripheral PA is effective in reducing pressure gradients and improving pulmonary blood flow. Indications for Intervention pulmonary angioplasty or stenting include symptoms attributed to decrease pulmonary blood flow, focal narrowing, abnormal differential perfusion, and/or elevated RV pressure. • Post procedure CNS Review with Bloods + ECHO within 6-8 weeks. • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor Post Intervention should be ordered. Follow-up • Physiological stage A - OPA with ACHD Team with ECG and ECHO every 24-36 months; Exercise Test every 36 months; CMR every 36-60 months. • Physiological stage B - OPA with ACHD Team with ECG and ECHO every 24 Long-term Follow- months; Exercise Test every 24 months; CMR every 36-60 months. up • Physiological stage C: OPA with ACHD Team with ECG and ECHO every 6-12 months; Exercise Test every 24 months; CMR every 24-36 months. • Physiological stage D - OPA with ACHD Team with ECG and ECHO every 3-6 months; Exercise Test every 12 months; CMR every 24-36 months. Issued: April 2019 Version: 001 Page | 84 Link - Contents Page 16.13 Double-Chambered Right Ventricle • Referral to Level 1 Centre • DCRV can be missed on TTE ECHO therefore CMR; CT; or Cardiac Catheter might be Confirm required to confirm diagnosis and establish the hemodynamic impact Diagnosis • Commonly associated with a VSD. Hypertrophied muscle bundles develop in the RV cavity, creating RVOT obstruction. • Chest X-ray; Blood test, ECG; CMR; MVO2; Assessment • If Echocardiogram and MRI differ - Cardiac Catheter may be required to assess for RV pressures and gradients. • Surgical repair for adults with DCRV and moderate or greater outflow obstruction is recommended in patients with otherwise unexplained symptoms of HF, cyanosis, or exercise limitation. Intervention • Surgical repair for adults with DCRV with a severe gradient may be considered in asymtomatic patients. • Surgical repair should contemplate closure of the VSD • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks. Post Intervention • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should Follow-up be ordered. • Physiological stage A - OPA with ACHD Team; ECG and ECHO every 24-36 months. (Exercise Test as required). • Physiological stage B - OPA with ACHD Team with ECG and ECHO every 24 months; Long-term Exercise Test every 24 months. Follow-up • Physiological stage C - OPA with ACHD Team every 6-12 months; ECG and ECHO every 12 months; Exercise Test every 24 months. • Physiological stage D - OPA with ACHD Team every 3-6 months; ECG and ECHO every 12 months; Exercise Test every 12 months. Issued: April 2019 Version: 001 Page | 85 Link - Contents Page 16.14 Right Ventricle to Pulmonary Artery Conduit • Referral to Level 1 Centre Confirm • TTE ECHO to assess conduit anatomy and function and also valve Diagnosis function. Assessmen • ECG, Chest Xray, Bloods, MVO2 and CMR. t • In patients with stented RV to PA conduits and worsening PS or PR, evaluation for conduit complications should be performed including fluoroscopy to evaluate for stent fracture and blood cultures to assess for IE. • In adults with RV to PA conduit and arrhythmia, congestive HF, unexplained ventricular dysfunction or cyanosis cardiac catheterisation is reasonable to assess hemodynamics • Coronary artery compression testing with simultaneous coronary angiography and high pressure dilation in the conduit is indictated before RV to PA conduit stenting or Interventio transcatheter valve replacement. n • RV to PA conduit intervention is reasonable for adults with RV to PA conduit and moderate or greater PR or moderate or greater stenosis with reduced functional capacity or arrhythmia; or in asymptomatic patients with severe stenosis or severe regurgitation with reduced ejection fraction or RV dilation. Post • Post procedure ECHO within 6-8 weeks. ECG to ensure sinus rhythm if concerned Interventio about arrhythmia holter monitor should be ordered. n Follow- up • Physiological stage A - OPA with ACHD Team with ECH and ECHO every 12-24 months; ECG and ECHO every 24 months; CMR every 36-60 months (Exercise Test as required). Long-term Follow-up • Physiological stage B - OPA with ACHD Team with ECG and ECHO every 12 months; CMR every 36-60 months (Exercise Test as required). • Physiological stage C - OPA with ACHD Team every 6-12 months; ECG and ECHO every 12 months; CMR and Exercise Test every 12-24 months. • Physiological stage D - OPA with ACHD Team every 3-6 months; ECG and ECHO every 12 months; CMR and Exercise Test every 12-24 months. Issued: April 2019 Version: 001 Page | 86 Link - Contents Page 16.15 Congenital Mitral Stenosis • Referral to Level 1 Centre Confirm Diagnosis • Chest X-ray; ECG; Bloods, CMR; MVO2; TTE ECHO. Stress echo might be required. • RH Catheter may be required to assess for any other associated defects. Assessmen • Complete assessment of other known associated congenital abnormalities, such as the t components of Shone Syndrome • Indication for valvce repair / replacement are as follows: • Significant mitrals tenosis (area <1.5 cm2) in symptomatic patients; if asymptomatic, surgery should be considered if: history of systemic embolism, dense spontaneous contrast in the left atrium, new-onset atrial fibrillation, systolic PAP >50 mmHg at rest, need for major non-cardiac surgery or desire for pregnancy. Interventio n • Choices and techniques for valve repair or replacement are based on consideration of coexisting abnormalities. • Congenital mitral stenosis is usually unsuitable for percutaneously baloon valvuloplasty. Post • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks. Interventio • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be n Follow-up ordered • Recurrence and progression of other congenital abormalities is expected. • Physiological Stage A - OPA with ACHD Team with ECG and ECHO every 24 months (Exercise test as required) • Physiological Stage B - OPA with ACHD Team with ECG and ECHO every 24 months (Exercise Long-term test as required) Follow-up • Physiological Stage C - OPA with ACHD Team every 6-12 months with ECG and ECHO every 12 months. Exercise test every 24 months. • Physiological Stage D - OPA with ACHD Team every 3-6 months with ECG and ECHO every 12 months. Exercise test every 12 months. Issued: April 2019 Version: 001 Page | 87 Link - Contents Page 16.16 Cor Triatriatum • Referral to Level 1 centre Confirm • Diagnosis should be confirmed confirmed via TTE ECHO. Diagnosis • Chest X-ray; Bloods, ECG; CMR; MVO2; • Complete assessment should be made for other congenital abnormalities particularly ASD, VSD, and anomalous pulmonary venous connection (total or partial). Assessment • Carefully rule out pulmonary venous stenosis - this has been demonstrated before and after surgery. • Cardiac Catheter may be required during assessment. • Intervention should be considered in adults with cortriatriatum and symptoms attributable to the obstruction OR when there is a substantial gradient across the membrane (suggested gradient is ≥8mmHg). Intervention • Intervention can be considered with gradient <8mmHg if symptoms and/or arrhythmia are present. • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks. • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be Post intervention ordered Follow-up • Follow-up is dependant on the presence of any other congenital abnormalities - if other abnormalities present then follow up should be according with the recommended follow-up for the congenital anomaly. • If no other congenital abnormalities are present should be based on the residual Long-term gradient. Follow-up • Follow-up arranged for 12-36 months with ECG and ECG - according to consultant ACHD cardiologist. • Exercise Test and CMR may be recommended during follow-up as per advice of consultant ACHD cardiologist. Issued: April 2019 Version: 001 Page | 88 Link - Contents Page 16.17 (Partial) Anomalous Pulmonary Venous Drainage • Referral to Level 1 Centre Confirm • Diagnosis of PAPVD confirmed on TTE ECHO Diagnosis • Chest X Ray, Bloods, MVO2 and ECG. Assessme • Cardiac MRI or CT Angiography are recommended for evaluation of PAPVD. nt • RH Cardiac Catheterisation can be used to further define haemodynamics. • Surgical intervention for PAPVD is recommended when impaired functional capacity and RV enlargement are present, there is a left to right shunt sufficient to cause physiological sequelae (i.e. Qp:Qs ≥1.5:1) with PAP <50% systemic pressure, and PVR <1/3 systemic resistance. Interventio n • If Sinus venosus ASD present, repair of PAPVD is recommended at time of Sinus venosus ASD closure. • Repair of Scimitar vein is recommended if functional capacity is impaired, evidence of RV volume overload is present, left to right shunt sufficiently large to cause physiological sequelae (i.e. Qp:Qs ≥1.5:1), PAP <50% systemic pressure, and PVR <1/3 systemic resistance. • In the absense of symptoms, it is reasonable to repair PAPVC and Scimitar vein if there are signs of RV volum overload and QP:Qs ≥1.5:1. Post • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks to assess baffling Interventio of veins to LA or anastomosis sites, and also to check for SVC obstruction. n Follow- • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be up ordered. • Physiological Stage A - OPA with ACHD Team with ECG and ECHO every 36-60 months (Pulse Oximetry and Exercise test as required) • Physiological Stage B - OPA with ACHD Team with ECG and ECHO every 24 months Long-term (Pulse Oximetry and Exercise test as required) Follow-up • Physiological Stage C - OPA with ACHD Team every 6-12 months with ECG and ECHO every 12 months. Pulse Oximetry at each visit and Exercise test every 12-24 months. • Physiological Stage D - OPA with ACHD Team every 3-6 months with ECG and ECHO every 12 months. Pulse Oximetry at each visit and exercise test every 6-12 months. Issued: April 2019 Version: 001 Page | 89 Link - Contents Page 16.18 Tetralogy of Fallot • Referral to Level 1 Centre Confir •Residual lesions should be first assessed by Echo TTE m Diagno sis • Chest X-ray; Blood test, ECG; MVO2. • CMR should be used to quantify ventricular size and function, pulmonary valve function, pulmonary artery anatomy, and left heart abnormalities. •Cardiac catheter with angiography is reasonable to assess hemodynamics when this cannot be assess non-invasively in the setting of arrhythmia, HF, unexplained ventricular dysfunction, suspected PHT or cyanosis. Assess ment •In patients with risk factors, consider refer to ARIA Clinic for SCD Risk Assessment •Programmed ventricular stimulation can be useful to risk-stratisfy adults with TOF and additional risk factors for sudden cardiac death. •Consider Genetic Testing for DiGeorge Sd (specially in women with pregnancy desire) • PVR (surgical or percutaneous) is indicated in moderate or greater PR in: • For the reflief of symptoms • In aymptomatic patients for preservation of ventricular size and function with ventricular enlargement or dysfunction (RVEDV ≥160ml/m2, RVESV ≥80ml/m2, or RVEDV≥ 2x LVEDV); if RVOTO with RVSP ≥2/3 systemic; if the exercice capacity is declining in the MVO2. Interve ntion • Surgical PVR is reasonable if there are other lesions requiring surgery. • PVR in addition to arrhythmia management may be considered for adults with repaired TOF and moderate or greater PR and ventricular tachycardia. • Coronary artery compression testing is indicated before RV to PA conduit stenting or transcatheter valve replacement in repaired TOF Post • Post intervention CNS Review with Bloods and ECHO within 6-8 weeks. Interve ntion • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be ordered. Follow- up Long- term Follow- up •Physiological stage A - OPA with ACHD Team every 12-24 months; ECG and ECHO every 24 months; CMR every 36 months; Exercise test every 36-60 months (Pulse oximetry and Holter monitor as required). •Physiological stage B - OPA with ACHD Team with ECG every 12 months; ECHO every 12-24 months; CMR every 24-36 months; Exercise Test every 24-60 months (Pulse Oximetry and Holter monitor as required). •Physiological stage C - OPA with ACHD Team with Pulse Oximetry every 6-12 months; ECG and ECHO every 12 months; CMR, Exercise Test and Holter monitor every 12-24 months. •Physiological stage D - OPA with ACHD Team with Pulse Oximetry every 3-6 months; ECHO every 6-12 months; ECG every 12 months; CMR, Exercise Tes, Holter monitor every 12-24 months. Issued: April 2019 Version: 001 Page | 90 Link - Contents Page 16.19 Ebstein Anomaly • Referral to Level 1 Centre Confirm Diagnosis • In adults with Ebstein Anomaly ECHO and CMR are useful to determine anatomy, RV dimensions and systolic function. • Request as baseline: Bloods, ECG, Chest X Ray and MVO2 (with Sat). Holter monitor if history of palpitations or syncopal episodes. Assessment • Electrophysiology study with or without catheter ablation can be useful in the diagnostic evaluation of adults with Ebstein anomaly. • Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present: • HF symptoms. • Objective evidence of worsening exercise capacity. Intervention • Progressive RV systolic dysfunction by ECHO or CMR. • Surgical repair or reoperation for adults with Ebstein anomaly and sig TR can be beneficial in the presence of: progressive RV enlargement, systemic desaturation from right to left atrial shunt, paradoximal embolism, and/or atrial tachyarrhythmia. • Post procedure CNS Review with Bloods + ECHO within 6-8 weeks to assess TR and RV function; Post Intervention Follow-up • ECG to ensure sinus rhythm. If concerned about arrhythmia holter monitor should be ordered. • Physiological stage A - OPA with ACHD Team with ECG and ECHO every 12-24 months; Pulse Oximetry every 24 months; Exercise Test every 36 months; CMR every 60 months Long-term Follow-up (CXR and Holter Monitor as required). • Physiological stage B - OPA with ACHD Team with ECG every 12 months; ECHO every 12-24 months; Pulse Oximetry every 12 months; Exercise Test every 24-36 months; CMR every 36 months (CXR and Holter Monitor as required). • Physiological stage C - OPA with ACHD Team with Pulse Oximetry every 6-12 months; ECG and ECHO every 12 months; CXR every 12-24 months; Exercise Test every 24 months; CMR every 24-36 months; Holter Monitor every 24 months. • Physiological stage D - OPA with ACHD Team with ECG and Pulse Oximetry every 3-6 months; ECHO every 12 months; Exercise Test every 12 months; CMR; Holter monitor and CXR every 12-24 months. Issued: April 2019 Version: 001 Page | 91 Link - Contents Page 16.20 Truncus arteriosus •Referral to level 1 Centre Confirm diagnosis •ECG, CXR, echocardiogram, CMR, cardio-pulmonary exercise test, routine bloods •Cardiac catheterization may be considered Assessment •Truncus arteriosus in the adult has almost invariably been repaired in childhood •In the rare circumstances when an adult has unrepaired truncus arteriosus, Eisenmenger physiology is typical; Pulmonary hypertension may also be present in repaired patients •The types of operative repairs may involve VSD closure, right ventricle–to-pulmonary artery conduit placement, reconstruction of the pulmonary arteries, and replacement of the truncal (neoaortic) valve Intervention •Unifocalization of the pulmonary arteries may be necessary in very complex cases •Recommendations regarding assessment and management of truncus arteriosus are inferred in the recommendations for the specific components, including right ventricle–to-pulmonary artery conduit, VSD, aortic valve disease, aortopathies and Eisenmenger physiology •Patients with Eisenmenger Physiology or Pulmonary Hypertension after repair should be Longterm referred to PH4 Clinic follow-up Issued: April 2019 Version: 001 Page | 92 Link - Contents Page 16.21 Double Outlet Right Ventricle • Referral to level 1 Centre Confirm diagnosis • ECG, CXR, echocardiogram, CMR, cardio-pulmonary exercise test, routine bloods • Cardiac catheterization may be considered • Liver imaging (Ultrasonography with elastography, CMR, CT) and laboratory evaluation of liver function for fibrosis, cirrhosis, and/or hepatocellular carcinoma is required in patients with single-ventricle physiology and Fontan Assessment palliation • Double Outlet Right Ventricle is an anatomic descriptor that includes various abnormalities. Types of repairs are predicated on the underlying anatomy and may involve VSD closure with relief of pulmonary stenosis, right ventricle–to- pulmonary artery conduit, or Rastelli-type repair. In some cases, single-ventricle Intervention physiology may be present and Fontan palliation as a result performed • Guidelines for the management of a patient with double outlet right ventricle are inferred in the recommendations for the lesion with the most similar Longterm anatomy and physiology follow-up Issued: April 2019 Version: 001 Page | 93 Link - Contents Page 16.22 Fontan •Referral to level 1 Centre Confirm diagnosis •ECG, CXR, echocardiogram, CMR, cardio-pulmonary exercise test, routine bloods •Liver imaging (Ultrasonography with elastography, CMR, CT) and laboratory evaluation of liver function for fibrosis, cirrhosis, and/or hepatocellular carcinoma Assessment •Cardiac catheterization may be required •New presentation of an atrial tachyarrhythmia in adults with Fontan palliation should be managed promptly and include prevention of thromboembolic events and consultation with an electrophysiologist with CHD expertise for a consideration of electrophysiology study and potential ablation •Anticoagulation with a vitamin K antagonist is recommended for adults with Fontan palliation with known or suspected thrombus, thromboembolic events, prior atrial arrhythmia, atriopulmonary Fontan connection and no contraindications to anticoagulation •In selected patients novel oral anticoagulant (NOAC) can be used if anticoagulation is recommended •Antiplatelet therapy or anticoagulation with a vitamin K antagonist may be considered in adults after Fontan palliation without known or suspected thrombus, thromboembolic Management events, or prior arrhythmia and with total cavo-pulmonary connection (lateral tunnel or extracardiac conduit) •Pulmonary vasoactive medications can be beneficial to improve exercise capacity in adults with Fontan repair •Heart failure medications i.e. angiotensin converting enzyme inhibitors, angiotensin receptor blockers and mineralocorticoid receptor antagonists may be used with a potential benefit in adults after Fontan palliation with cardiac dysfunction Issued: April 2019 Version: 001 Page | 94 Link - Contents Page •Fontan revision surgery, including arrhythmia surgery as indicated, may be considered for adults with atriopulmonary Fontan connections with recurrent atrial tachyarrhythmias refractory to pharmacological therapy and catheter ablation who have preserved systolic ventricular function, no valvular disease, low pulmonary artery pressures (mPAP<15mmHg) and severe atrial dilation •Reoperation or intervention for structural/ anatomic abnormalities in a Fontan palliated patient with symptoms or with failure of the Fontan circulation may be considered Intervention •Heart transplant may be considered in Fontan patients with cardiac dysfunction, Fontan pathway dysfunction, lymphatic dysfunction or extracardiac dysfunction as specified in heart transplant referral pathway •Physiological Stage A - OPA with ACHD Team with ECG and ECHO every 12 months, CMR/CT every 36months, Cardiopulmonary eercise Test every 36 months •Physiological Stage B - OPA with ACHD Team with ECG and ECHO every 12 months, CMR/CT every 24months, Exercise test every 24 months. •Physiological Stage C - OPA with ACHD Team with ECG every 6months, ECHO every 12 months. CMR /CT every 24 months, Cardiopulmonary exercise test every 12 months. •Physiological Stage D - OPA with ACHD Team withh ECG every 3-6 months , ECHO every 12 months, CMR/CT every 24 months, ECardiopulmonary eercise test every 12 months. •Imaging of the liver (ultrasonography with elastography, CMR, CT) and laboratory evaluation of liver function for fibrosis, cirrhosis, and/or hepatocellular carcinoma every 1-2 years in adults after Longterm Fontan palliation •Bochemical and hematological testing every 12 months especially for liver and renal function follow-up •Cardiac catheterization should be performed in adults before initial Fontan surgery or revision of a prior Fontan connection to assess suitability of preintervention hemodynamics for Fontan physiology or revision of a prior Fontan connection •New onset or worsening atrial tachyarrhythmias in adults with single ventricle after Fontan palliation should prompt a search for potential hemodynamic abnormalities, which may necessitate imaging and/or cardiac catheterization •Cardiac catheterization can be useful to evaluate a symptomatic adult after Fontan palliation when noninvasive testing is insufficient to guide therapy •New presentation of an atrial tachyarrhythmia in adults with Fontan palliation should prompt consultation with an electrophysiologist with CHD expertise Issued: April 2019 Version: 001 Page | 95 Link - Contents Page 16.23 Transposition of the Great Arteries – Atrial Switch • Referral to Level 1 Centre Confirm Diagnosis • Chest X-ray; Blood test, ECG; MVO2 • TTE Echocardiogram and CMR to assess specially RV size and function, tricuspid valve function, subpulmonic obstruction, obstruction of systemic or pulmonary venous return, and baffle leaks. Assessment • Holter monitor should be considered, as there is a progressive loss of sinus rhythm, particularly in the context of beta-blocker treatment. • Atrial tachycardias should be addressed immediately, as they can be a potential cause for SCD in these patients. • Efforts to maintain sinus rhythm or atrial pacing (and not simply rate control) should be the Intervention initial strategy • Assessment of SCD risk and ICD implantation should be considered in collaboration with the EP team • Post intervention CNS Review with Bloods and ECHO within 6-8 weeks. Post Intervention • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be Follow-up ordered. • Physiological stage A - OPA with ACHD Team with ECG and pulse oximetry every 12 months; ECHO every 12-24 months; Holter monitor every 24 months; CMR every 24-36 months; Exercise Test every 36 months; • Physiological stage B - OPA with ACHD Team with ECG and pulse oximetry every 12 months; ECHO every 12-24 months; Holter monitor 24 months; CMR every 24 months; Exercise Test every 36 months; • Physiological stage C - OPA with ACHD Team with ECG and pulse oximetry every 6-12 Long-term months; ECHO every 12 months; Holter monitor every 12 months; CMR every 12-24 Follow-up months; Exercise Test every 24 months. • Physiological stage D - OPA with ACHD Team with ECG and pulse oximetry every 3-6 months; ECHO every 12 months; Holter monitor every 12 months; CMR every 12-24 months; Exercise Test every 12 months. • Patients with RV dysfunction and HF symptoms should be referred for Heart Transplant Assessment. Issued: April 2019 Version: 001 Page | 96 Link - Contents Page 16.24 Transposition of the Great Arteries – Arterial Switch •Referral to Level 1 Centre Confirm Diagnosis •Chest X-ray; Blood test, TTE Echocardiogram, ECG; CMR; MVO2 •Baseline assessment of the coronary arteries with CTCA/coronary catheterization should be performed Assessment •Physiological exams of myocardial perfusion (like stress echo, nuclear test) should be performed in symptomatic patients • Coronary revascularization should be based on the current Revasc Guidelines and the Interventional Team should be envolved • Complications of the arterial switch, such as RVOT obstruction (supravalvular PS), might Intervention require catheter or surgical intervention if symptoms of HF or decreased exercise capacity are present. • Dilatation of neoaorta or signficant aortic regurgitation might need to be adressed surgically. •Post procedure CNS Review with Bloods and ECHO within 6-8 weeks. Post Intervention •ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be Follow-up ordered. •Physiological stage A - OPA with ACHD Team with ECG and ECHO every 12-24 months; Exercise Test and MRI every 36 -60 months months; •Physiological stage B - OPA with ACHD Team with ECG every 12 months; ECHO every 12- Long-term 24 months; CMR every 24-36 months; Exercise Test every 36-60 months; Follow-up •Physiological stage C - OPA with ACHD Team ECG every 6-12 months; ECG and ECHO every 12 months; CMR every 12-24 months; Exercise Test every 24-36months. •Physiological stage D - OPA with ACHD Team with ECG every 3-6 months; ECHO every 12 months; CMR and Exercise Test every 12 months; Issued: April 2019 Version: 001 Page | 97 Link - Contents Page 16.25 Congenitally Corrected Transposition of the Great Arteries • Referral to Level 1 Centre Confirm Diagnosis • Chest X-ray; Blood test, ECG; CMR; MVO2 • TTE Echocardiogram, specially oriented on the RV size and function and tricuspid valve anatomy and function. Assessment • Holter monitor should be considered, as the prevalence of spontaneous complete heart block increases with age. • Triscupid valve replacement (or repair in selected patients) is indicated in symptomatic patients with severe TR and preserved (or only mildly impaired) RV impairment. • In asymptomatic patients, tricuspid valve replacement can be considered in patients with Intervention severe TR, RV dilatation and mild RV dysfunction. • In patients with LV-PA conduit dysfunction, conduit replacement might be considered (but unloading the LV might negatively impact the RV function). • Post surgery CNS Review with Bloods and ECHO within 6-8 weeks. Post Intervention • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor should be Follow-up ordered. • Physiological stage A - OPA with ACHD Team with ECG and ECHO every 12 months; Holter monitor every 12-60 months; Exercise Test and CMR every 36 -60 months months; Pulse oximetry as needed. • Physiological stage B - OPA with ACHD Team with ECG and ECHO every 12 months; Holter monitor every 12-60 months; Exercise Test and CMR every 36 -60 months months; Pulse oximetry as needed. • Physiological stage C - OPA with ACHD Team with ECG and pulse oximetry every 6-12 Long-term months; ECHO every 12 months; Holter monitor every 12-36 months; CMR and Exercise Follow-up Test every 12-24 months. • Physiological stage D - OPA with ACHD Team with ECG and pulse oximetry every 3-6 months; ECHO every 12 months; Holter monitor every 12 months; CMR and Exercise Test every 12 months. •Patients with RV dysfunction and HF symptoms should be referred for Heart Transplant Assessment. Issued: April 2019 Version: 001 Page | 98 Link - Contents Page 16.26 Anomalous Aortic Origin of Coronary Artery • Referral to Level 1 Centre Confirm Diagnosis •CTCA should be performed to help identify high risk anatomic features like fish-mouth- shaped or slit-like orifice, or intramural course. •Physiological study should be performed with a Stress Echo Assessment •Other basic investigations: Chest X-ray; Blood test, TTE Echo, ECG; CMR; MVO2 • Coronary catheterization (with IVUS) might be required. •Surgical repair for adults should be considered in the presence of ischemia symptoms or if the stress test is positive for ischemia or arrythymias Intervention •Surgery or continued observation may be reasonable for asymptomatic patients without ischemia or anatomic or physiological evaluation suggesting potential for compromise • Post procedure CNS Review with Bloods and ECHO within 6-8 weeks. Post Intervention • ECG to ensure sinus rhythm if concerned about arrhythmia holter monitor Follow-up should be ordered. Long-term •Every 12 months (with functional test) if not repaired Follow-up •Every 24-36 months (with functional test if appropriate) if repaired. Issued: April 2019 Version: 001 Page | 99 Link - Contents Page 16.27 PH and Eisenmenger Syndrome • Referral to Level 1 Centre •Patients should be assessed by the ACHD and PH Team Confirm Diagnosis • Chest X-ray; Blood test, ECG; MVO2 or 6 min walking test • TTE Echocardiogram and CMR to assess specially RV size and function. • RHC to confirm pulmonary vascular hemodynamics and to exclude other potential Assessment contributors to right-to-left shunting or pulmonary hypertension. Intervention • Therapeutic treatment is indicated in patients with FC II, III or IV WHO • Patients with Pulmonary Hypertension or Eisenmenger Syndrom should be referred to PH4 Clinic. • Physiological stage C - OPA with ACHD Team with ECG and pulse oximetry every 6-12 months; ECHO every 12 months; CMR as needed; Exercise Test (CPET or 6 min WT) every 6- 12 months. Long-term • Physiological stage D - OPA with ACHD Team with ECG and pulse oximetry every 3-6 Follow-up months; ECHO every 12 months; CMR as needed; Exercise Test (CPET or 6 min WT) every 6- 12 months. • Patients with HF symptoms should be referred for Heart and Lung Transplant Assessment. • Cyanotic patients --> Endocarditis Prophylaxis for invasive dental procedures is recommended 16.28 Reference Stoute et al. 2018 AHA / ACC Guideline for the Management of Adults with Congenital Heart Disease. Circulation. 2019; 139. Issued: April 2019 Version: 001 Page | 100 Link - Contents Page