DOCSLIB.ORG

It's My Heart

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text

Load more

It’s My Heart The World’s Leading Guide to Congenital Heart Defects for Patients and Parents Revised Edition www.childrensheartfoundation.org (888) 248-8140 Copyright © 2011 by The Children’s Heart Foundation All rights reserved, including the right to reproduce this book or portions thereof in any form whatsoever. ISBN: 0-9841447-9-2 Table of Contents Introduction I. The Normal Heart Development of the Normal Heart Anatomy and Function of the Normal Heart Types of Abnormal Heart Disease II. Congenital Heart Defects Aortic Stenosis Atrial Septal Defect (ASD) Atrioventricular Septal Defect (AVSD) Coarctation of the Aorta Congenitally Corrected Transposition of the Great Arteries Double Inlet Left Ventricle (DILV) Double Outlet Right Ventricle (DORV) Ebstein’s Malformation Hypoplastic Left Heart Mitral Valve Abnormalities Patent Ductus Arteriosus Pulmonary Atresia With Intact Ventricular Septum Pulmonary Atresia With Ventricular Septal Defect (VSD) Pulmonary Stenosis Tetralogy of Fallot (TOF) Total Anomalous Pulmonary Venous Return (TAPVR) Transposition of the Great Arteries Tricuspid Atresia Truncus Arteriosus,Types I-IV Ventricular Septal Defect (VSD) III. Acquired Heart Conditions Arrhythmia Cardiomyopathy Congestive Heart Failure Endocarditis Pericarditis Rheumatic Heart Disease Viral Myocarditis IV. Diagnostic Tests Cardiac Catheterization Chest X-ray Computerized Axial Tomography (CT Scan) Echocardiogram Electrocardiogram (ECG/EKG) Electrophysiology Studies (EP Studies) Holter Monitor Magnetic Resonance Imaging (MRI) Prebirth Testing Stress Testing V. Equipment Chest Tube Central IV Lines Defibrillator System Endotracheal Tube (ET Tube) Foley Catheter Heart-Lung Bypass Machine Heart Monitoring Equipment Nasogastric Tube (NG Tube) Oxygen Pacemaker System Pulse Oximeter Suction Procedure Temporary Pacemaker Ventilator/Respirator Ventricular Assist Devices VI. Surgical Procedures Arterial Switch Blalock-Hanlon Procedure Blalock-Taussig Shunt Closure of Septal Defects Correction of Narrow Valves Damus-Kaye-Stansel Operation Double Switch Operation Fontan Operations Glenn Shunt Hybrid Procedure Kawashima Operation Ligation (Division) of Patent Ductus Arteriosus Mustard Procedure Norwood Procedures,Type 1 and 2 One-and-One-Half Ventricle Repair Pulmonary Artery Banding Rastelli Procedure Repair of Anomalous Pulmonary Venous Return Repair of Coarctation of the Aorta Repair of Common Atrio-Ventricular (AV) Canal Repair of Tetralogy of Fallot Ross Procedure VII. Medications ACE Inhibitors/Vasodilators Anticoagulants Antiarrhythmics Beta Blockers Diuretics Experimental Drugs Inotropic Drugs Potassium Supplements VIII. Congenital Heart Disease in the Adult General Issues Preventative Measures for Healthy Lifestyle Activity/Exercise Preventing Endocarditis Regularly Established Plan for Follow-up of Heart Problem Hospitalization and/or ER Visit Travel Considerations Lifestyle Issues Reproductive Issues IX. Family Living Bacterial Endocarditis Dental Care Issues CPR Training and Renewal Fire Department Notification Preparing for Daycare, School, Camp, etc. X. Preparing for Hospitalization Discussing Hospitalization Preparing the Infant Preparing the Toddler Preparing the Preschooler Preparing the School Age Child Preparing the Adolescent Preparing Yourselves Preparing for Discharge after a Medical Admission Preparing for Discharge after a Surgical Admission At Home Following a Hospitalization Sibling Issues Know What’s Best for Your Family Glossary Additional Resources Introduction It’s My Heart This free resource is for patients and families affected by congenital and acquired heart disease. It is a guide to be used with medical professionals. It is not a substitute for your child’s physician. Never hesitate to direct any questions or concerns about your child’s heart problem to your pediatric cardiologist... no question is too simple or silly. You know your child better than anyone. Don’t be shy about calling your child’s physician to discuss any concerns. If your child looks different, acts different, or is having difficulty eating, always call. Also, know that you are not alone. Approximately 40,000 babies are born in the United States each year with congenital heart defects. That represents about one in every 125 births, topping the list as America’s most prevalent birth defect. There are many ways in which the heart can develop abnormalities. Genetic and environmental factors have been linked to congenital heart disease. But in most cases, the cause is unknown. The good news is that most people who have childhood heart surgery grow up to be healthy adults. But it is important to get the heart care that you need to keep well. If your heart defect is listed as complex, experts recommend that you get life-long care from experts in congenital heart disease. This is true even if you had successful surgery in childhood. It’s My Heart is intended to provide you with information on some of the many issues you may be dealing with since your child was diagnosed with a congenital heart defect. The book was written by doctors, nurses and parents in a collaborative effort spearheaded by The Children’s Heart Foundation (CHF) in Lincolnshire, Illinois. Neither the book nor the Foundation are directly associated with any one hospital or institution. The National Board of Directors of The Children’s Heart Foundation wishes to thank the following individuals for their commitment and generosity in providing information, time and resources to the development of It’s My Heart: It’s My Heart Project Committee: Thomas Weigel, M.D.; Carl Backer, M.D.; Marla Mendelson, M.D.; Hilton Hudson, M.D.; Julie Creaden, CPNP; Betsy Peterson, CHF Founder; Jena Rausch, publisher; Barbra Armaroli, first-edition editor; and CHF staff members Rosemary Wheeler, Christina Larson and Ellen Weiss. Chapter Authors and Illustrators: Alexander J. Muster, M.D.; Katherine A. Tubeszewski, RN, MSN; Lynn C. Graham, RN, BSN; Katherine Kerrigan, RN, MS, CPNP; Maggie Fischer, RN, MS; Jennifer Cleary, RN, MS; Linda Hellstedt, MSN, RN; Sue Rushmore; Natalie Rudolph, RN, MSN; Betsy Peterson; illustrations are used by permission of John Wiley & Sons, Inc.; Congenital Heart Disease: A Diagrammatic Atlas by Charles E. Mullins, M.D. and David C. Mayer, M.D., Copyright © 1988 WileyLiss, Inc.; Medtronic, Inc. The Children’s Heart Foundation also wishes to thank the Northwestern University Dance Marathon 2011 for its generous support of the second edition of the book, as well as Medtronic Foundation for its support for funding the initial production and distribution of this book; the Gerber Foundation, WNUA Cares for Kids Foundation, Circle of Service Foundation, Inc., and the Winnetka Rotary for funding the development of the book; Amanda Haley for the beautiful and unique It’s My Heart logo; Andrea Wilhelm, AHW Design, for layout and design; Angela Vennemann for updated layout; and Hilton Hudson, M.D., and the team at Hilton Publishing, Inc. The Children’s Heart Foundation was founded in 1996. Its mission is to raise funds to support research toward discovering the causes of and improving the methods of diagnosing, treating and preventing congenital heart defects. The Children’s Heart Foundation is a 501(c)(3) tax-exempt charitable organization. CHF is not affiliated with any one medical institution or organization. This allows funds to be directed to the most promising research anywhere in the world, under the direction of our Medical Advisory Board. About this Book When the first edition of It’s My Heart was published in 2004, it addressed a dire need noted by patients with congenital heart defects and their parents and families. Although doctors and nurses often explained things well, written materials were either too simple (pamphlets on how the normal heart works) or too complex (medical journals). What was needed was a book with clear writing and illustrations on the most common kinds of heart defects, tests, equipment, surgical procedures and medications. It’s My Heart was the answer to that need, and it became an immediate success! Notable is CHF’s decision from the start to make the book available free for families and to post the entire contents of the book on its website for free reading or downloading. Countless people around the world have benefited from the book in its English and Spanish versions. For this revised edition of It’s My Heart, the entire contents have been reviewed and, where needed, updated with the latest in medical information. Contact CHF at www.childrensheartfoundation.org or (888) 248-8140 for further information on the organization’s activities throughout the United States. To view a current roster of CHF’s Medical Advisory Board, visit www.childrensheartfoundation.org/about-chf/officers-boards-staff. The Normal Heart The heart has two roles. First, it delivers oxygen-rich blood to all living tissues in the body. Second, it recirculates used blood (in which the oxygen has been replaced by carbon dioxide) through the lungs, where carbon dioxide is exhaled and replaced by oxygen that has been inhaled. Arteries carry blood away from the heart and veins return blood to the heart. Development of the Normal Heart and Vessels During the first seven weeks after conception, known as the embryonic period, the heart completes development from a single straight tube into a complicated four-chambered pump containing four valves. From then until the baby is born, the fetal period, the heart and the vessels grow in size. Until a newborn takes its first breath after clamping
Recommended publications
  • The Conversion to Rastelli's Type Operation from Patrick-Mcgoon's

    The Conversion to Rastelli's Type Operation from Patrick-Mcgoon's

    General Thoracic and Cardiovascular Surgery (2019) 67:551–553 https://doi.org/10.1007/s11748-018-0942-x CASE REPORT The conversion to Rastelli’s type operation from Patrick-McGoon’s procedure of an adult with Taussig–Bing heart: a case report Keiichi Fujiwara1 · Kousuke Yoshizawa1 · Nobuhisa Ohno1 · Hisanori Sakazaki2 Received: 30 January 2018 / Accepted: 18 May 2018 / Published online: 11 June 2018 © The Japanese Association for Thoracic Surgery 2018 Abstract A 23-year-old female of Taussig–Bing heart with antero-posterior relation of the great arteries was underwent Patrick- McGoon’s intraventricular rerouting at 6 years old of age. The left ventricular outflow obstruction (peak pressure gradient of 100 mmHg) developed, and severe aortic valve regurgitation following bacterial endocarditis was noted. The conversion to Rastelli’s type operation and aortic valve replacement were performed successfully at 23 years old of age. She is doing well without any significant left or right ventricular outflow obstruction at 7 years postoperatively. Keywords Taussig–Bing heart · Intraventricular rerouting · Patrick-McGoon’s operation · Left ventricular outflow obstruction · Adult congenital heart disease Introduction Case Taussig–Bing heart is characterized by double-outlet right A 23-year-old female diagnosed of double outlet right ventricle with subpulmonary ventricular septal defect [1, 2]. ventricle with subpulmonary ventricular septal defect and Currently, the arterial switch procedure as anatomic repair, antero-posterior relation of the great arteries, was referred to connecting the left ventricle to aorta and the right ventricle our hospital for severe left ventricular outflow tract obstruc- to pulmonary artery, is a popular surgical management for tion following Patrick-McGoon’s intraventricular rerouting Taussig–Bing heart regardless of relation of the great arter- and moderate to severe aortic valve regurgitation following ies.
  • Cardiology Today Jan-Feb 2019.Pdf

    Cardiology Today Jan-Feb 2019.Pdf

    VOLUME XXIII No. 1 JANUARY-FEBRUARY 2019 PAGES 1-40 Rs. 1700/- ISSN 0971-9172 RNI No. 66903/97 www.cimsasia .com Cardiology MANAGING DIRECTOR & PUBLISHER Dr. Monica Bhatia TODAY EDITOR IN CHIEF OP Yadava SECTION EDITORS SR Mittal (ECG, CPC), David Colquhou n (Reader’s Choice) EDITORIAL NATIONAL EDITORIAL ADVISORY BOARD Circadian Rhythm of the Body - Is it the Holy Arun K Purohit, Arun Malhotra, Ashok Seth, Grail ? 3 Ashwin B Mehta, CN Manjunath, DS Gambhir, OP YADAVA GS Sainani, Harshad R Gandhi, I Sathyamurthy, Jagdish Hiremath, JPS Sawhney, KK Talwar, K Srinath Reddy, KP Misra, ML Bhatia, Mohan Bhargava, MR Girinath, Mukul Misra, Nakul Sinha, PC Manoria, Peeyush Jain, Praveen Jain, Ramesh Arora, Ravi R Kasliwal, S Jalal, S Padmavati, Satyavan Sharma, SS Ramesh, Sunil Kumar Modi, Yatin Mehta, Yogesh Varma, R Aggarwala. INTERNATIONAL EDITORIAL ADVISORY BOARD REVIEW ARTICLE Andrew M Tonkin, Bhagwan Koirala, Carlos A Mestres, Chuen N Lee, David M Colquhoun, Davendra Mehta, Contrast Induced Nephropathy: How to Enas A Enas, Gerald M Pohost, Glen Van Arsdell, Indranill Basu Ray, James B Peter, James F Benenati, Predict and Prevent? 5 Kanu Chatterjee, Noe A Babilonia, Pascal R Vouhe, RAGHAV BANSAL, VIVEKA KUMAR Paul A Levine, Paul Simon, P K Shah, Prakash Deedwania, Salim Yusuf, Samin K Sharma, Sanjeev Saxena, Sanjiv Kaul, Yutaka Imoto. DESK EDITOR Gandhali DESIGNER A run Kharkwal REVIEW ARTICLE OFFICES CIMS Medica India Pvt Ltd How do I Manage My Patients with Heart (Previously known as UBM Medica India Pvt Ltd.) Failure with Preserved Ejection Fraction? 10 Registered Office MOHAMMED SADIQ AZAM, DAYASAGAR RAO V Margosa Building, No.
  • Cardiac Arrhythmias Following the Creation Ofan Atrial Septal Defect in Patients with Transposition of the Great Arteries

    Cardiac Arrhythmias Following the Creation Ofan Atrial Septal Defect in Patients with Transposition of the Great Arteries

    Thorax: first published as 10.1136/thx.28.2.147 on 1 March 1973. Downloaded from Thorax (1973), 28, 147. Cardiac arrhythmias following the creation of an atrial septal defect in patients with transposition of the great arteries R. J. MOENE, J. P. ROOS, and A. EYGELAAR Departments of Paediatric Cardiology and Cardiology, Free University Hospital, Amsterdam, and the Department of Thoracic Surgery, University Hospital, Groningen, The Netherlands In 64 children with transposition of the great arteries who underwent a Blalock-Hanlon pro- cedure, pre- and postoperative electrocardiograms were studied regarding the incidence and nature of rhythm disturbances. In another group of 19 patients with transposition of the great arteries, the atrial septal defect was created by a different surgical technique (fossa ovalis resection); this group was studied in the same way and the results were compared. After the Blalock-Hanlon procedure seven patients developed arrhythmias including atrio- ventricular (A-V) dissociation, wandering pacemaker, bradycardia, atrial flutter, supraventricular tachycardia, supraventricular premature beats, and ectopic atrial rhythm. After fossa ovalis resection rhythm disturbances were present in three patients. Despite their relatively high incidence it seems unlikely that arrhythmias are a major factor copyright. contributing to death irrespective of the technique used; some arrhythmias are transient and serious disturbances of long duration are rare. In complete transposition of the great arteries the tion) using temporary
  • Dye Dilution Curves After the Artificial Atrial Septostomy in Three Infants with the Transposition of the Great Vessels

    Dye Dilution Curves After the Artificial Atrial Septostomy in Three Infants with the Transposition of the Great Vessels

    Pohoku J. exp. Med., 1970, 100, 39-46 Dye Dilution Curves after the Artificial Atrial Septostomy in Three Infants with the Transposition of the Great Vessels Hiroshi Onoki, Tetsuo Sato, Ichiki Kano and Keiko Mochizuki Department of Pediatrics (Prof. Ts. Arakawa), Faculty of Medicine, Tohoku University, Sendai Hemodynamic consequences after the Rashkind and Miller balloon atrial septostomy were successfully evaluated by means of the dye dilution technic in three infants with the complete transposition of the great vessels. The complete transposition of the great vessels has been the most common cause of death in infants born with congenital malformations of the heart; accord ing to Boesen1 forty-two per cent of the patients with this anomaly succumbed within one month of life and seventy-three per cent within the first three months of life. In 1964 Mustard2 reported a case of the transposition of the great vessels in which the successful result of the radical operation was obtained by adopting a two-stage correction technic; that is, creation of an atrial septal defect was done by Blalock-Hanlon's procedure with thoracotomy when 20 days of life, then successful radical operation was carried out at the age of 18 months. In 1966 Rashkind and Miller3 devised a method for the creation of an atrial septal defect without thoracotomy as a palliative approach to the complete transposi tion of the great vessels. Then in 1968, Rashkind and Miller4 reported thirty-one infants with the transposition of the great vessels who were subjected to the balloon atrial septostomy of theirs with a marked improvement in the interatrial blood commu nication.
  • The Arterial Switch Operation for Transposition of the Great Arteries

    The Arterial Switch Operation for Transposition of the Great Arteries

    The Arterial Switch Operation for Transposition of the Great Arteries Richard A. Jonas Transposition of the great arteries, together with tetral- Ijased on the fact that (luring fetal life hoth ventricles ogy of Fallot, is one of the most common congenital are exposed to the same pressure load. Thus, the left cardiac anomalies resulting in cyanosis. It is hardly ventricle is prepared at birth. However, hecause pulnio- surprising, therefore, that shortly after the introduc- nary resistance falls ra1)idlj within 6 to 8 weeks ancl tion of cardiopulmonary hypass in the early 195Os, perhaps as quicltly as within 2 to 4 weeks, the left attempts were made at anatomical correction of transpo- ventricle is no longer prepared. Iiitroduction of an sition, ie, the arterial switch procedure. These early essentially elective complex neonatal operation in 1983 attempts were uniformly unsuccessful for a number of initially met with consitlerahle resistance, particularly reasons : 1)ecause hy this time the surgical mortality for the atrial 1. Microvascular techniques were not adequately level procedures had reached very low levels. However, developed for delicate coronary artery transfer. a prospectivr study conducted Ijy the Congenital Heart 2. Pulmonary vascular disease is common in transpo- Surgeons Society‘ showed that the overall survival of an sition beyond the first year of life. entire population of patients with transposition en- 3. Surgeons failed to appreciate that in the presence rolled in the neonatal period was superior with the of an intact ventricular septum, the left ventricle was neonatal arterial switch approach because of the previ- inadequately prepared to acntely take over the pressure ously uncaptured deaths that occurred before atrial load of the systemic circulation.
  • Tricuspid Atresia Associated with Common Arterial Trunk and 22Q11 Chromosome Deletion

    Tricuspid Atresia Associated with Common Arterial Trunk and 22Q11 Chromosome Deletion

    Archivos de Cardiología de México Volumen Número Octubre-Diciembre Volume 73 Number 4 October-December 2003 Artículo: Tricuspid atresia associated with common arterial trunk and 22q11 chromosome deletion Derechos reservados, Copyright © 2003 Instituto Nacional de Cardiología Ignacio Chávez Otras secciones de Others sections in este sitio: this web site: ☞ Índice de este número ☞ Contents of this number ☞ Más revistas ☞ More journals ☞ Búsqueda ☞ Search edigraphic.com 271 COMUNICACIONES BREVES Tricuspid atresia associated with common arterial trunk and 22q11 chromosome deletion Carlos Alva,* Felipe David,* Martha Hernández,* Rubén Argüero,** José Ortegón,* Arturo Martínez,* Diana López,* Santiago Jiménez,* Agustín Sánchez* Summary Resumen The case of a four-months old male with coexist- ATRESIA TRICUSPÍDEA Y TRONCO ARTERIOSO COMÚN ent tricuspid atresia and common arterial trunk is presented. The diagnois was made by cardiac Se presenta el caso de un paciente masculino catheterization and selective angiocardiography. de cuatro meses de edad con atresia tricuspí- Clinical considerations are discussed and the dea asociada a tronco arterial común. El diag- review of the available literature reveals this pa- nóstico se corroboró con cateterismo cardíaco y tient to be the tenth case reported of this very angiocardiografía selectiva. Se analiza la expre- unusual association of cardiovascular defects, sión clínica de esta excepcional asociación y se and the first with positive deletion of the 22q11 hace una revisión de la literatura. Este es el dé- chromosome. cimo caso publicado con esta inusual asociación de lesiones cardíacas y el primero con deleción del cromosoma 22q11. (Arch Cardiol Mex 2003; 73:271-274). Key words: Tricuspid atresia and common arterial trunk.
  • World Journal for Pediatric & Congenital Heart Surgery

    World Journal for Pediatric & Congenital Heart Surgery

    World Journal for Pediatric & Congenital Heart Surgery Keywords List A __________________________________________________________________________________ Ablative therapy (all modalities) Acute respiratory distress syndrome (ARDS) Adult Adult Congenital Heart Disease Airway Allograft, homograft Anastomosis Anatomy Anesthesia (includes agents, pharmacology, care and research) Aneurysm (aortic, myocardial, other) Angiogenesis Angiography Angioplasty Angioplasty (balloon dilatation) Animal model Annuloplasty (indicate location) Antibiotics Antibody/antigen Aorta/aortic Aortic arch Aortic dissection (includes ulcers, hematomas) Aortic operation Aortic root Aortic valve, repair Aortic valve, replacement Apoptosis Arrhythmia Arrhythmia surgery (includes Maze) Arterial switch operation Artery/arteries Artificial organs Atherosclerosis Atrial fibrillation, flutter Atrial Septal Defect (ASD) Atrio-ventricular Septal Defect (AVSD) Atrium Autograft Autonomic nervous system B __________________________________________________________________________________ Biochemistry Bioengineering Biomaterials Biopsy (indicate tissue) Blood Blood conservation Blood substitutes Blood transfusion Blood, coagulation/anticoagulation Brain Bronchial arteries Bronchiolitis obliterans Bronchoscopy/bronchus C __________________________________________________________________________________ Calcification Cardiac (use in combination) Cardiac anatomy/pathologic anatomy Cardiac catheterization/intervention Cardiac function Cardiac tumors (includes myxoma, primary, metastases)
  • Atrial Septal Stenting to Increase Interatrial Shunting in Cyanotic Congenital Heart Diseases: a Report of Two Cases

    Atrial Septal Stenting to Increase Interatrial Shunting in Cyanotic Congenital Heart Diseases: a Report of Two Cases

    422 Türk Kardiyol Dern Arş - Arch Turk Soc Cardiol 2011;39(5):422-426 doi: 10.5543/tkda.2011.01368 Atrial septal stenting to increase interatrial shunting in cyanotic congenital heart diseases: a report of two cases Siyanotik doğuştan kalp hastalıklarında interatriyal şantı artırmak amacıyla atriyal septuma stent uygulaması: İki olgu sunumu Yalım Yalçın, M.D., Cenap Zeybek, M.D.,§ İbrahim Özgür Önsel, M.D.,# Mehmet Salih Bilal, M.D.† Departments of Pediatric Cardiology, #Anesthesiology and Reanimation, and †Cardiovascular Surgery, Medicana International Hospital; §Department of Pediatric Cardiology, Şişli Florence Nightingale Hospital, İstanbul Summary – Aiming to increase mixing at the atrial level, Özet – Siyanotik doğuştan kalp hastalığı tanısıyla izle- atrial septal stenting was performed in two pediatric nen iki bebekte, atriyal düzeyde karışımı artırmak ama- cases with cyanotic congenital cardiac diseases. The cıyla atriyal septuma stent yerleştirme işlemi uygulandı. first case was a 3-month-old male infant with transpo- Birinci olgu, büyük arterlerin transpozisyonu tanısıyla sition of the great arteries. The second case was an izlenen üç aylık bir erkek bebekti. Diğer olgu, ameliyat 18-month-old male infant with increased central venous sonrası dönemde sağ ventrikül çıkım yolu tıkanıklığına pressure due to postoperative right ventricular outflow bağlı olarak santral venöz basınç yüksekliği gelişen 18 tract obstruction. Premounted bare stents of 8 mm in aylık bir erkek bebekti. Her iki olguda da 8 mm çapında, diameter were used in both cases. The length of the balona monte edilmiş çıplak stent kullanıldı. Stent uzun- stent was 20 mm in the first case and 30 mm in the lat- luğu ilk olguda 20 mm, ikinci olguda 30 mm idi.
  • Tricuspid Valve Guideline

    Tricuspid Valve Guideline

    Tricuspid Valve What the Nurse Caring for a Patient with Congenital Heart Disease Needs to Know Lindsey Justice, DNP, RN, CPNP-AC, Nurse Practitioner, Cardiac Intensive Care Unit, Cincinnati Children’s Hospital Medical Center Justine Mize, MSN, RN, CCRN, CPN, Professional Practice Specialist, Cardiac Intensive Care Unit, Children’s National Health System, Washington, DC Louise Callow, MSN, RN, CPNP, Nurse Practitioner, Pediatric Cardiac Surgery, University of Michigan, CS Mott Children’s Hospital, Ann Arbor, Michigan Mary Rummell, MN, RN, CPNP, CNS, FAHA Clinical Nurse Specialist, Pediatric Cardiology/Cardiac Services, Oregon Health & Science University (Retired) Embryology Occurrence: o Defects of cardiac valves are the most common subtype of cardiac malformations o Account for 25% to 30% of all congenital heart defects o Most costly and relevant CHD o Wise spectrum of congenital defects in tricuspid valve Development of the heart valves occurs during the fourth to eighth weeks of gestation- after tubular heart looping o Walls of the tubular heart consist of an outer lining of myocardium and an inner lining of endocardial cells o Cardiac jelly, extensive extracellular matrix (ECM), separates the two layers o Cardiac jelly expands to form cardiac cushions at the sites of future valves . Outflow track (OT) valves = aortic and pulmonic valves Final valves derived from endothelial-mesenchymal cells with neural crest cells from the brachial arches Valves (Semilunar) have 3 equal cusp-shaped leaflets Aortic valve incorporates coronary arteries . Atrioventricular (AV) valves = mitral and tricuspid Final valves derived entirely from endocardial cushion tissue Leaflet formed without a cusp Two leaflets associated with left ventricle (mitral) Three leaflets associated with right ventricle (tricuspid) Coordinated by complex interplay of: o Genetics o Signaling pathways that regulate cell apoptosis and proliferation o Environmental factors 1 .
  • Congenitally Corrected Transposition Gonzalo a Wallis1*, Diane Debich-Spicer2,3 and Robert H Anderson4

    Congenitally Corrected Transposition Gonzalo a Wallis1*, Diane Debich-Spicer2,3 and Robert H Anderson4

    Wallis et al. Orphanet Journal of Rare Diseases 2011, 6:22 http://www.ojrd.com/content/6/1/22 REVIEW Open Access Congenitally corrected transposition Gonzalo A Wallis1*, Diane Debich-Spicer2,3 and Robert H Anderson4 Abstract Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum.
  • Appendix A: Surgical Procedure Terms and Definitions

    Appendix A: Surgical Procedure Terms and Definitions

    Appendix A: Surgical Procedure Terms and Definitions Anomalous Systemic Venous Connection Anomalous Systemic Venous Connection Repair Repair includes a range of surgical approaches, including, among others: ligation of anomalous vessels, reimplantation of anomalous vessels (with or without use of a conduit), or redirection of anomalous systemic venous flow through directly to the pulmonary circulation (bidirectional Glenn to redirect LSVC or RSVC to left or right pulmonary artery, respectively). Aortic Aneurysm Aortic aneurysm repair Aortic aneurysm repair by any technique. Aortic Dissection Aortic Dissection repair Aortic dissection repair by any technique. Aortic Root Replacement Aortic Root Replacement, Bioprosthetic Replacement of the aortic root (that portion of the aorta attached to the heart; it gives rise to the coronary arteries) with a bioprosthesis (e.g., porcine) in a conduit, often composite. Aortic Root Replacement, Mechanical Replacement of the aortic root (that portion of the aorta attached to the heart; it gives rise to the coronary arteries) with a mechanical prosthesis in a composite conduit. Aortic Root Replacement, Homograft Replacement of the aortic root (that portion of the aorta attached to the heart; it gives rise to the coronary arteries) with a homograft Aortic Root Replacement, Valve sparing Replacement of the aortic root (that portion of the aorta attached to the heart; it gives rise to the coronary arteries) without replacing the aortic valve (using a tube graft). Aortic Valve Disease Ross Procedure Replacement of the aortic valve with a pulmonary autograft and replacement of the pulmonary valve with a homograft conduit. Konno Procedure (with and without aortic valve replacement) Relief of left ventricular outflow tract obstruction associated with aortic annular hypoplasia, aortic valvar stenosis and/or aortic valvar insufficiency via Konno aortoventriculoplasty.
  • Fellow in Training Research Abstracts Oral Competition Indiana-ACC Poster Competition Abstract

    Fellow in Training Research Abstracts Oral Competition Indiana-ACC Poster Competition Abstract

    Fellow in Training Research Abstracts Oral Competition Indiana-ACC Poster Competition Abstract Do NOT write outside the boxes. Any text or images outside the boxes will be deleted. Do NOT alter this form by deleting parts of it (including this text) or adding new boxes. Please structure your clinical research abstract using the following headings: * Background * Objective * Methods * Results (if relevant) * Conclusion Please structure your case study abstract using the following headings: * Introduction/objective * Case presentation * Discussion * Conclusion Title: Multi-Disciplinary Team Approach to Cardiogenic Shock Reduces In-Hospital Mortality Abstract: (Your abstract must use Normal style and must fit into the box. You may not alter the size of this ) Purpose: To determine the effectiveness of a hospital-wide process improvement program labeled as level 1 cardiogenic shock rapid response that enables a concerted coordinated multi-disciplinary team approach to treatment of patients with cardiogenic shock. Methods: Conducted a retrospective analysis of the cardiogenic shock rapid response program (protocol group) comparing to similar cohort of cardiogenic shock patients who did not utilize the shock response program. The program required alerting the advanced heart failure trained cardiologist who advises activation of a burst page to the cardiogenic shock team. This team rapidly institutes protocol-directed therapy and use of advanced support devices if necessary. The primary endpoint was hospital mortality. Secondary endpoint was utilization of advanced therapy (temporary mechanical circulatory support) Results: 471 patients were in the control and 110 in the protocol group. Baseline characteristics were similar and etiology of cardiogenic shock. The protocol group had significant reductions in-hospital mortality (35% to 45% (p< 0.05).