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2020 ESC Guidelines for the Management of Adult Congenital ESC GUIDELINES European Heart Journal (2020) 00,1À83 doi:10.1093/eurheartj/ehaa554 2020 ESC Guidelines for the management of adult congenital heart disease Downloaded from https://academic.oup.com/eurheartj/advance-article/doi/10.1093/eurheartj/ehaa554/5898606 by guest on 29 August 2020 The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC) Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD) Authors/Task Force Members: Helmut Baumgartner* (Chairperson) (Germany), Julie De Backer* (Chairperson) (Belgium), Sonya V. Babu-Narayan (United Kingdom), Werner Budts (Belgium), Massimo Chessa 1 (Italy), Gerhard-Paul Diller (Germany), Bernard Iung (France), Jolanda Kluin (Netherlands), Irene M. Lang (Austria), Folkert Meijboom (Netherlands), Philip Moons (Belgium), Barbara J. M. Mulder (Netherlands), Erwin Oechslin (Canada), Jolien W. Roos-Hesselink (Netherlands), Markus Schwerzmann (Switzerland), Lars Sondergaard (Denmark), Katja Zeppenfeld (Netherlands) Document reviewers: Sabine Ernst (CPG Review Coordinator) (United Kingdom), Magalie Ladouceur (CPG Review Coordinator) (France), Victor Aboyans (France), David Alexander (United Kingdom), Ruxandra Christodorescu (Romania), Domenico Corrado (Italy), Michele D’Alto (Italy), * Corresponding authors: Helmut Baumgartner, Department of Cardiology III: Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert Schweitzer Campus 1, Building A1, D-48149, Muenster, Germany. Tel: þ49 251 83 46110, Fax: þ49 251 83 46109, E-mail: [email protected]. Julie De Backer, Department of Cardiology, Ghent University Hospital, C. Heymanslaan 10, 9000 Ghent. Tel: þ32 9 332 56 27, E-mail: [email protected]. ESC Committee for Practice Guidelines (CPG) and National Cardiac Societies document reviewers, and Author/Task Force Member Affiliations: listed in the Appendix. 1Representing the AEPC. ESC entities having participated in the development of this document: Associations: Association for Acute Cardiovascular Care (ACVC), Association of Cardiovascular Nursing & Allied Professions (ACNAP), European Association of Cardiovascular Imaging (EACVI), European Association of Preventive Cardiology (EAPC), European Association of Percutaneous Cardiovascular Interventions (EAPCI), European Heart Rhythm Association (EHRA), Heart Failure Association (HFA). Councils: Council for Cardiology Practice, Council of Cardio-Oncology, Council on Valvular Heart Disease. Working Groups: Adult Congenital Heart Disease, Aorta and Peripheral Vascular Diseases, Cardiovascular Pharmacotherapy, Cardiovascular Surgery, Development Anatomy and Pathology, e-Cardiology, Pulmonary Circulation and Right Ventricular Function. The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC ([email protected]). Disclaimer. The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge, and the evidence avail- able at the time of their publication. The ESC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the ESC Guidelines and any other offi- cial recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the ESC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of pre- ventive, diagnostic, or therapeutic medical strategies; however, the ESC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient’s health condition and in consultation with that patient and, where appropriate and/or necessary, the patient’s caregiver. Nor do the ESC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient’s case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription. VC The European Society of Cardiology 2020. All rights reserved. For permissions please email: [email protected]. 2 ESC Guidelines Natasja de Groot (Netherlands), Victoria Delgado (Netherlands), Giovanni Di Salvo (United Kingdom), Laura Dos Subira (Spain), Andreas Eicken1 (Germany), Donna Fitzsimons (United Kingdom), Alexandra A. Frogoudaki (Greece), Michael Gatzoulis (United Kingdom), Stephane Heymans (Netherlands/Belgium), Ju¨ rgen Ho¨ rer (Germany), Lucile Houyel (France), Guillaume Jondeau (France), Hugo A. Katus (Germany), Ulf Landmesser (Germany), Basil S. Lewis (Israel), Alexander Lyon (United Kingdom), Christian E. Mueller (Switzerland), Darren Mylotte (Ireland), Steffen E. Petersen (United Kingdom), Anna Sonia Petronio (Italy), Marco Roffi (Switzerland), Raphael Rosenhek (Austria), Evgeny Shlyakhto (Russia), Iain A. Simpson (United Kingdom), Miguel Sousa-Uva (Portugal), Christian Tobias Torp-Pedersen (Denmark), Rhian M. Downloaded from https://academic.oup.com/eurheartj/advance-article/doi/10.1093/eurheartj/ehaa554/5898606 by guest on 29 August 2020 Touyz (United Kingdom), Alexander Van De Bruaene (Belgium) The disclosure forms of all experts involved in the development of these Guidelines are available on the ESC website www.escardio.org/guidelines ................................................................................................................................................................................................... Keywords Guidelines • congenital heart disease • disease • adult • diagnosis • imaging • late complications • medical treatment • congenital cardiac surgery • catheter intervention • patient follow-up • recommendations . 3.4.3.3 Therapeutic management of pulmonary hypertension Table of contents . in adult congential heart disease ............................... 21 . 3.4.3.3.1 Expert centres ................................... 21 1Preamble......................................................... 6 . 3.4.3.3.2 General measures ................................ 21 2Introduction...................................................... 8 . 3.4.3.3.3 Anticoagulation .................................. 21 2.1 Why do we need new Guidelines on the management of . 3.4.3.3.4 Shunt repair ..................................... 22 adultcongenitalheartdisease?.................................... 8 . 3.4.3.3.5 Pulmonary arterial hypertension-directed medical 2.2ContentoftheseGuidelines .................................. 8 . therapy .................................................. 22 2.3NewformatoftheGuidelines................................. 8 . 3.4.4Surgicaltreatment....................................... 22 2.4HowtousetheseGuidelines.................................. 8 . 3.4.5Catheterintervention ................................... 23 2.5Whatisnewinthe2020Guidelines? .......................... 8 . 3.4.6Infectiveendocarditis.................................... 23 3Generalaspects .................................................. 8 . 3.4.7Antithrombotictreatment............................... 23 3.1 Prevalence of adult congenital heart disease . 8 . 3.4.8Managementofcyanoticpatients......................... 23 3.2Organizationofcare.......................................... 8 . 3.4.8.1 Adoptive mechanisms ................................. 24 3.3Diagnosticwork-up ......................................... 13 . 3.4.8.2 Multisystem disorder .................................. 24 3.3.1Echocardiography....................................... 15 . 3.4.8.3 Clinical presentation and natural history ................. 24 3.3.2 Cardiovascular magnetic resonance imaging . 16 . 3.4.8.4 Late complications .................................... 24 3.3.3 Cardiovascular computed tomography . 16 . 3.4.8.5 Diagnostic aspects .................................... 24 3.3.4Cardiopulmonaryexercisetesting ....................... 16 . 3.4.8.6 Laboratory precautions ................................ 25 3.3.5Cardiaccatheterization.................................. 16 . 3.4.8.7 Indications for intervention ............................. 25 3.3.6Biomarkers ............................................. 16 . 3.4.8.8 Medical therapy ...................................... 25 3.4Therapeuticconsiderations .................................. 17 . 3.4.8.9 Follow-up recommendations ........................... 25 3.4.1Heartfailure ............................................ 17 . 3.4.8.10 Additional considerations ............................. 26 3.4.2 Arrhythmias and sudden cardiac death . 17 . 3.5Additionalconsiderations...................................
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