Laurence IserinLaurianne Le Gloan

DIU de Cardiologie pédiatrique et congénitaleParis, le 17 mars 2017

Cardiopathies congénitales de l’adulte

ESC GUIDELINES

ESC Guidelines for the managementof grown-up congenital heart disease(new version 2010)The Task Force on the Management of Grown-up Congenital HeartDisease of the European Society of Cardiology (ESC)Endorsed by the Association for European Paediatric Cardiology (AEPC)Authors/Task Force Members: Helmut Baumgartner (Chairperson) (Germany)*, Philipp Bonhoeffer (UK),Natasja M. S. De Groot (The Netherlands), Fokko de Haan (Germany), John Erik Deanfield (UK), Nazzareno Galie(Italy), Michael A. Gatzoulis (UK), Christa Gohlke-Baerwolf (Germany), Harald Kaemmerer (Germany),Philip Kilner (UK), Folkert Meijboom (The Netherlands), Barbara J. M. Mulder (The Netherlands), Erwin Oechslin(Canada), Jose M. Oliver (Spain), Alain Serraf (France), Andras Szatmari (Hungary), Erik Thaulow (Norway),Pascal R. Vouhe (France), Edmond Walma (The Netherlands).

ESC Committee for Practice Guidelines (CPG): Alec Vahanian (Chairperson) (France), Angelo Auricchio(Switzerland), Jeroen Bax (The Netherlands), Claudio Ceconi (Italy), Veronica Dean (France), Gerasimos Filippatos(Greece), Christian Funck-Brentano (France), Richard Hobbs (UK), Peter Kearney (Ireland), Theresa McDonagh(UK), Bogdan A.Popescu (Romania), ZeljkoReiner (Croatia), UdoSechtem (Germany), Per AntonSirnes (Norway),Michal Tendera (Poland), Panos Vardas (Greece), Petr Widimsky (Czech Republic).

Document Reviewers: Theresa McDonagh (CPG Review Coordinator) (UK), Lorna Swan (Co-Review Coordinator)(UK), FelicitaAndreotti (Italy), MauriceBeghetti (Switzerland), Martin Borggrefe (Germany), Andre Bozio (France),StephenBrecker (UK), WernerBudts (Belgium), JohnHess (Germany), RafaelHirsch (Israel), GuillaumeJondeau(France), JormaKokkonen (Finland), Mirta Kozelj (Slovenia), SerdarKucukoglu (Turkey), Mari Laan (Estonia),ChristosLionis (Greece), IrakliMetreveli (Georgia), Philip Moons (Belgium), Petronella G.Pieper (The Netherlands),VladimirPilossoff (Bulgaria), Jana Popelova (Czech Republic), Susanna Price (UK), JolienRoos-Hesselink (TheNetherlands), Miguel SousaUva (Portugal), PilarTornos (Spain), Pedro TrigoTrindade (Switzerland), HeikkiUkkonen(Finland), HamishWalker (UK), Gary D.Webb (USA), JørgenWestby (Norway).

The disclosure forms of the authors and reviewers are available on the ESC website www.escardio.org/guidelines

ESC entities having participated in the development of this document:Associations: European Association of Percutaneous Cardiovascular Interventions (EAPCI), European Heart RhythmAssociation (EHRA), Heart Failure Association (HFA), European Association of Echocardiography (EAE)Councils: Cardiology Practice, Council on Primary Care, Cardiovascular Imaging, Cardiovascular Nursing and AlliedProfessions (CCNAP)Working Groups: Grown-up Congenital Heart Disease, Pulmonary Circulation and Right Ventricular Function, ValvularHeart Disease, Cardiovascular Surgery, Thrombosis, Acute Cardiac Care

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 theESC 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 OxfordUniversity Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC.

* Corresponding author. Adult Congenital and Valvular Heart Disease Center (EMAH-Zentrum) Muenster, Department of Cardiology and Angiology, University Hospital Muenster,Albert-Schweitzer-Str. 33, D-48149 Muenster, Germany. Tel: +49 251 8346110, Fax: +49 251 8346109, Email: helmut.baumgartner@ukmuenster.de

Disclaimer. The ESC Guidelines represent the views of the ESC and were arrived at after careful consideration of the available evidence at the time they were written. Healthprofessionals are encouraged to take them fully into account when exercising their clinical judgement. The guidelines do not, however, override the individual responsibility of healthprofessionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient, and where appropriate and necessary the patient’sguardian or carer. It is also the health professional’s responsibility to verify the rules and regulations applicable to drugs and devices at the time of prescription.

& The European Society of Cardiology 2010. All rights reserved. For permissions please email: journals.permissions@oxfordjournals.org

European Heart Journaldoi:10.1093/eurheartj/ehq249

Baumgartner et al. Eur Heart J 2010

Stout et al. JACC 2018

MANUSCRIPT

ACCEPTED MANUSCRIPTStout KK, et al. 2018 ACHD Guideline

Page 1

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease

A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease,

Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

WRITING COMMITTEE MEMBERS* Karen K. Stout, MD, FACC, Chair†

Curt J. Daniels, MD, Vice Chair*†‡

Jamil A. Aboulhosn, MD, FACC, FSCAI*§ Stephanie Fuller, MD, MS, FACC#

Biykem Bozkurt, MD, PhD, FACC, FAHA║ Michelle Gurvitz, MD, FACC**

Craig S. Broberg, MD, FACC*† Paul Khairy, MD, PhD*†

Jack M. Colman, MD, FACC† Michael J. Landzberg, MD, FACC*†

Stephen R. Crumb, DNP, AACC† Arwa Saidi, MB, BCH, FACC*†

Joseph A. Dearani, MD, FACC¶ Anne Marie Valente, MD, FACC, FAHA, FASE††

George F. Van Hare, MD‡‡

ACC/AHA TASK FORCE MEMBERS Glenn N. Levine, MD, FACC, FAHA, Chair

Patrick T. O’Gara, MD, MACC, FAHA, Chair-Elect Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair

Nancy M. Albert, PhD, RN, FAHA§§ Zachary D. Goldberger, MD, MS, FACC, FAHA

Sana M. Al-Khatib, MD, MHS, FACC, FAHA Mark A. Hlatky, MD, FACC

Joshua A. Beckman, MD, MS, FAHA John Ikonomidis, MD, PhD, FAHA

Kim K. Birtcher, PharmD, MS, AACC José Joglar, MD, FACC, FAHA

Biykem Bozkurt, MD, PhD, FACC, FAHA§§ Richard J. Kovacs, MD, FACC, FAHA§§

Ralph G. Brindis, MD, MPH, MACC§§ Laura Mauri, MD, MSc, FAHA

Joaquin E. Cigarroa, MD, FACC E. Magnus Ohman, MD, FACC§§

Lesley H. Curtis, PhD, FAHA§§ Mariann R. Piano, RN, PhD, FAHA, FAAN

Anita Deswal, MD, MPH, FACC, FAHA Susan J. Pressler, PhD, RN, FAHA§§

Lee A. Fleisher, MD, FACC, FAHA Barbara Riegel, PhD, RN, FAHA

Federico Gentile, MD, FACC Frank W. Sellke, MD, FACC, FAHA§§

Samuel S. Gidding, MD, FAHA§§ Win-Kuang Shen, MD, FACC, FAHA§§

Duminda N. Wijeysundera, MD, PhD

*Writing committee members are required to recuse themselves from voting on sections to which their specific

relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA Representa`ve.

‡Interna`onal Society for Adult Congenital Heart Disease Representa`ve. §Society for Cardiovascular Angiography

and Interventions Representative. ║ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of

Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task

PACES/HRS Expert Consensus Statement on theRecognition and Management of Arrhythmias in AdultCongenital Heart DiseaseDeveloped in partnership between the Pediatric and Congenital Electrophysiology Society (PACES)and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS,the American College of Cardiology (ACC), the American Heart Association (AHA),the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS),and the International Society for Adult Congenital Heart Disease (ISACHD)

Paul Khairy, MD, PhD, FRCPC (Chair),* George F. Van Hare, MD, FACC, FHRS (Co-Chair),†

Seshadri Balaji, MBBS, PhD,† Charles I. Berul, MD, FHRS,† Frank Cecchin, MD, FACC,‡

Mitchell I. Cohen, MD, FACC, FHRS,† Curt J. Daniels, MD, FACC,** Barbara J. Deal, MD, FACC,†

Joseph A. Dearani, MD, FACC,* Natasja de Groot, MD, PhD,¶ Anne M. Dubin, MD, FHRS,†

Louise Harris, MBChB, FHRS,# Jan Janousek, MD, PhD,¶ Ronald J. Kanter, MD, FHRS,†

Peter P. Karpawich, MD, FACC, FAHA, FHRS,† James C. Perry, MD, FACC, FHRS,*

Stephen P. Seslar, MD, PhD,† Maully J. Shah, MBBS, FHRS,† Michael J. Silka, MD, FACC, FAHA,§

John K. Triedman, MD, FACC, FHRS,† Edward P. Walsh, MD, FACC, FHRS,†

Carole A. Warnes, MD, FRCP, FACC, FAHA**

TABLE OF CONTENTS

Preamble ............................................................................... e1031. Methodology and evidence ........................................... e1032. Document review and approval .................................. e1053. Epidemiology and scope of arrhythmias in adults

with CHD ............................................................................ e1053.1. Changing mortality .............................................. e1053.2. Spectrum of arrhythmias ................................... e1053.3. Heart failure and arrhythmogenesis .............. e1063.4. Systemic right ventricle and univentricular

heart ........................................................................... e107

4. Delivery of care and ensuring access to care .............. e1084.1. Recommendations for the coordination and

delivery of care for adults with CHD andarrhythmias ............................................................. e108

4.2. Recommendations for adults with CHDrequiring invasive electrophysiologicinterventions ........................................................... e109

5. Evaluation and diagnosis of arrhythmias ................ e1105.1. Introduction ............................................................ e1105.2. General rhythm assessment based on

cardiac history and symptom status .............. e1105.3. Approach to the symptomatic patient .......... e1105.4. Approach to the asymptomatic patient ........ e113

6. Medical therapy ................................................................. e1156.1. Atrial tachyarrhythmias ..................................... e1156.2. Ventricular tachyarrhythmias .......................... e121

7. Catheter ablation ............................................................... e1227.1. General considerations for catheter ablation

in adults with CHD ............................................. e1227.2. AV reciprocating tachycardia and AV

nodal reentrant tachycardia .............................. e1227.3. Atrial tachyarrhythmias ..................................... e1237.4. Atrial fibrillation ................................................... e1247.5. Recommendations for catheter ablation of

atrial tachycarrhythmias in adultswith CHD ................................................................ e125

7.6. Ventricular tachycardia ...................................... e125

KEYWORDS Adult congenital heart disease; Congenital heart disease (HeartRhythm 2014;11:e102–e165)

Address reprint requests and correspondence: Dr. Paul Khairy, AdultCongenital Heart Center, Montreal Heart Institute, 5000 Belanger St. E.,Montreal, QC, Canada, H1T 1C8. E-mail address: paul.khairy@umontreal.ca.

*Pediatric and Congenital Electrophysiology Society (PACES) representa-

tive; †Heart Rhythm Society (HRS) representative; ‡American College of

Cardiology (ACC) representative; §American Heart Association (AHA)

representative; ¶European Heart Rhythm Association (EHRA) representa-

tive; #Canadian Heart Rhythm Society (CHRS) representative; **Interna-

tional Society for Adult Congenital Heart Disease (ISACHD) representative

1547-5271/$-see front matter B 2014 Heart Rhythm Society. All rights reserved. http://dx.doi.org/10.1016/j.hrthm.2014.05.009

Khairy et al. Heart Rhythm 2014

CURRENT OPINION

Treatment of heart failure in adult congenitalheart disease: a position paper of the WorkingGroup of Grown-Up Congenital Heart Diseaseand the Heart Failure Association of the EuropeanSociety of CardiologyWerner Budts1*, Jolien Roos-Hesselink2, Tanja Radle-Hurst3, Andreas Eicken4,Theresa A. McDonagh5, Ekaterini Lambrinou6, Maria G. Crespo-Leiro7, Fiona Walker8,and Alexandra A. Frogoudaki9

1Congenital and Structural Cardiology, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium; 2Department of Cardiology, Erasmus Medical Center Rotterdam,Rotterdam, The Netherlands; 3Department of Pediatric Cardiology, Saarland University Medical Center, Homburg, Germany; 4Deutsches Herzzentrum Munchen, Munich, Germany;5Department of Cardiology, King’s College Hospital, London, UK; 6Department of Nursing, School of Health Sciences Cyprus University of Technology, Limassol, Cyprus; 7AdvancedHeart Failure and Heart Transplantation Unit, Cardiology Service, Hospital Universitario A Coruna, La Coruna, Spain; 8Centre for Grown-Up Congenital Heart Disease, StBartholomews Hospital, London, UK; and 9Adult Congenital Heart Clinic, Second Cardiology Department, ATTIKON University Hospital and Athens University, Athens, Greece

Received 9 September 2015; revised 5 November 2015; accepted 14 December 2015; online publish-ahead-of-print 18 January 2016

Heart failure in congenital heartdisease: prevalence and outcomeImproved medical care of congenital heart disease patients in-creased survival into adulthood from 15% in the 1960s to over85% in the current era. As a consequence, the prevalence of adultcongenital heart disease (ACHD) increased rapidly,1 which is esti-mated to be .1 million ACHD patients in North America and1.2 million in Europe. The growing number and aging of ACHD pa-tients led to an overall increase in hospitalizations over the last dec-ade and a substantial increase in patients presenting with heartfailure (HF) (!20%).2

The incidence of first HF-admission was 1.2 per 1000 patient-years in the Dutch national ‘CONCOR’ registry. Patients admittedwith HF had a five-fold higher risk of death than those not admitted.From the same registry, the mortality was 2.8% during a follow-upperiod of 24 865 patient-years. Chronic HF (26%) and sudden death(19%) were recorded most frequently. The median age at deathfrom HF was 51.0 years (range: 20.3–91.2 years).3 In anotherACHD cohort, sudden death (26%) was the most common causeof death, followed by progressive HF (21%) and perioperative death(18%).4 Although patients with ACHD may not readily report symp-toms, clinical HF is documented in 22.2% of patients with a Mustardrepair for transposition of the great arteries (TGAs), 32.3% with

congenitally corrected transposition of the great arteries (ccTGA),and 40% of patients after Fontan palliation.

Pathophysiology of heart failurein adult congenital heart disease

Heart failure with impaired systolicventricular functionThe aetiology and triggers of impaired systolic ventricular function inACHD patients are summarized in Table 1.

Heart failure with preserved systolicventricular functionThis occurs less often in ACHD patients, but is associated with spe-cific conditions such as Shone complex and restrictive right ven-tricular (RV) physiology in the context of pulmonary atresia,ventricular septal defect (VSD), and major aorto-pulmonary collat-eral arteries.

Genetic and neurohormonal backgroundHeart failure in ACHD is the result not only of a structural defectbut also of defective contractility or conduction. An intriguing andemerging hypothesis is that genes involved in morphogenesis during

The opinions expressed in this article are not necessarily those of the Editors of the European Heart Journal or of the European Society of Cardiology.

*Corresponding author. Tel: +32 16 344369, Fax: +32 16 344240; Email: werner.budts@uzleuven.be

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2016. For permissions please email: journals.permissions@oup.com.

European Heart Journal (2016) 37, 1419–1427doi:10.1093/eurheartj/ehv741

Budts et al. Eur Heart J 2016AHA Scientific Statement

1

Part I: General ConsiderationsIntroductionThe past 60 years have brought remarkable advancements in the diagnosis and treatment of congenital heart disease (CHD). Early diagnosis and improvements in cardiac surgery and interventional cardiology have resulted in unprecedented survival of patients with CHD, even those with the most com-plex lesions. Despite remarkable success in treatments, many interventions are palliative rather than curative, and patients often develop cardiac complications, including heart failure (HF). HF management in the setting of CHD is challenged by the wide range of ages at which HF occurs, the heterogene-ity of the underlying anatomy and surgical repairs, the wide spectrum of HF causes, the lack of validated biomarkers for disease progression, the lack of reliable risk predictors or sur-rogate end points, and the paucity of evidence demonstrating treatment efficacy.

The purposes of this statement are to review the literature pertaining to chronic HF in CHD and to elucidate important gaps in our knowledge, emphasizing the need for specific stud-ies of HF mechanisms and improving outcomes for those with HF. In this document, the definition of CHD severity is the def-inition common in CHD documents, including the American College of Cardiology (ACC)/American Heart Association (AHA) guidelines1 for the management of adults with CHD

(Table 11–3). The definition of HF corresponds to that found in the multiple guidelines on diagnosis and management of HF. Although nuances and specific details may be controversial,4 the broad definition from the Heart Failure Society of America guidelines states the following: “In physiologic terms, HF is a syndrome characterized by either or both pulmonary and sys-temic venous congestion and/or inadequate peripheral oxygen delivery, at rest or during stress, caused by cardiac dysfunc-tion.”5 The definition of chronic HF in this document concurs with that of the European Society of Cardiology guidelines, which emphasize chronic HF (whether stable, progressively worsening, or decompensated) rather than acute HF. Although specific definitions of acute and chronic HF are not univer-sally accepted, we focus here on chronic HF as a persistent syndrome that requires consideration of therapy to prevent progression, decompensation, or death.4

This document focuses on the mechanisms and treat-ment of myocardial dysfunction while recognizing that HF symptoms may be attributable to underlying hemodynamic abnormalities such as valve dysfunction, outflow obstruc-tion, coronary abnormalities, or residual shunting. Therefore, all patients with CHD with HF symptoms should undergo a detailed hemodynamic assessment by CHD-experienced cardiologists for any reversible hemodynamic abnormali-ties and receive appropriately targeted interventions if pos-sible. Treatment recommendations for HF caused by valve

(Circulation. 2016;133:00-00. DOI: 10.1161/CIR.0000000000000352.)© 2016 American Heart Association, Inc.

Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000352

The American Heart Association makes every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.

This statement was approved by the American Heart Association Science Advisory and Coordinating Committee on January 15, 2015, and the American Heart Association Executive Committee on August 25, 2015. A copy of the document is available at http://my.americanheart.org/statements by selecting either the “By Topic” link or the “By Publication Date” link. To purchase additional reprints, call 843-216-2533 or e-mail kelle.ramsay@wolterskluwer.com.

The American Heart Association requests that this document be cited as follows: Stout KK, Broberg CS, Book WM, Cecchin F, Chen JM, Dimopoulos K, Everitt MD, Gatzoulis M, Harris L, Hsu DT, Kuvin JT, Law Y, Martin CM, Murphy AM, Ross HJ, Singh G, Spray TL; on behalf of the American Heart Association Council on Clinical Cardiology, Council on Functional Genomics and Translational Biology, and Council on Cardiovascular Radiology and Imaging. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133:XXX–XXX. doi: 10.1161/CIR.0000000000000352.

Expert peer review of AHA Scientific Statements is conducted by the AHA Office of Science Operations. For more on AHA statements and guidelines development, visit http://my.americanheart.org/statements and select the “Policies and Development” link.

Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the express permission of the American Heart Association. Instructions for obtaining permission are located at http://www.heart.org/HEARTORG/General/Copyright-Permission-Guidelines_UCM_300404_Article.jsp. A link to the “Copyright Permissions Request Form” appears on the right side of the page.

Chronic Heart Failure in Congenital Heart DiseaseA Scientific Statement From the American Heart Association

Karen K. Stout, MD, Chair; Craig S. Broberg, MD, Co-Chair; Wendy M. Book, MD; Frank Cecchin, MD; Jonathan M. Chen, MD; Konstantinos Dimopoulos, MD, MSc;

Melanie D. Everitt, MD; Michael Gatzoulis, MD, PhD; Louise Harris; Daphne T. Hsu, MD, FAHA; Jeffrey T. Kuvin, MD, FAHA; Yuk Law, MD;

Cindy M. Martin, MD; Anne M. Murphy, MD, FAHA; Heather J. Ross, MD, MHSc; Gautam Singh, MD; Thomas L. Spray, MD, FAHA; on behalf of the American Heart Association Council on Clinical Cardiology, Council on Functional Genomics and Translational Biology, and

Council on Cardiovascular Radiology and Imaging

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Stout et al. Circulation 2016

2018 ESC Guidelines for the management ofcardiovascular diseases during pregnancy

The Task Force for the Management of CardiovascularDiseases during Pregnancy of the European Society ofCardiology (ESC)

Endorsed by: the International Society of Gender Medicine (IGM),the German Institute of Gender in Medicine (DGesGM), theEuropean Society of Anaesthesiology (ESA), and the EuropeanSociety of Gynecology (ESG)

Authors/Task Force Members: Vera Regitz-Zagrosek* (Chairperson) (Germany),Jolien W. Roos-Hesselink* (Co-Chairperson) (The Netherlands), Johann Bauersachs(Germany), Carina Blomstrom-Lundqvist (Sweden), Renata C!ıfkov!a (CzechRepublic), Michele De Bonis (Italy), Bernard Iung (France), Mark Richard Johnson(UK), Ulrich Kintscher (Germany), Peter Kranke1 (Germany), Irene Marthe Lang(Austria), Joao Morais (Portugal), Petronella G. Pieper (The Netherlands),Patrizia Presbitero (Italy), Susanna Price (UK), Giuseppe M. C. Rosano (UK/Italy),Ute Seeland (Germany), Tommaso Simoncini2 (Italy), Lorna Swan (UK),Carole A. Warnes (USA)

* Corresponding authors. Vera Regitz-Zagrosek, Charite Universitaetsmedizin Berlin, Institute for Gender in Medicine, CCR, DZHK, partner site Berlin, Hessische Str 3-4, 10115Berlin, Germany, Tel: þ49 30 450 525 288, Fax: þ49 30 450 7 525 288, E-mail: vera.regitz-zagrosek@charite.de. Jolien W. Roos-Hesselink, Department of Cardiology, ErasmusMedical Center Rotterdam, Dr Molewaterplein 40, 3015CGD, Rotterdam, Netherlands, Tel: þ31 10 7032432, E-mail: j.roos@erasmusmc.nl

ESC Committee for Practice Guidelines (CPG) and National Cardiac Societies document reviewers: listed in the Appendix.

1Representing the European Society of Anaesthesiology (ESA)2Representing the European Society of Gynecology (ESG)

ESC entities having participated in the development of this document:

Associations: Acute Cardiovascular Care Association (ACCA), European Association of Cardiovascular Imaging (EACVI), European Association of Percutaneous CardiovascularInterventions (EAPCI), European Heart Rhythm Association (EHRA), Heart Failure Association (HFA).

Councils: Council on Cardiovascular Nursing and Allied Professions, Council on Cardiovascular Primary Care, Council on Hypertension, Council on Valvular Heart Disease.

Working Groups: Aorta and Peripheral Vascular Diseases, Cardiovascular Pharmacotherapy, Cardiovascular Surgery, Grown-up Congenital Heart Disease, Myocardial andPericardial Diseases, Pulmonary Circulation and Right Ventricular Function, Thrombosis.

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 theESC 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 OxfordUniversity Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC (journals.permissions@oxfordjournals.org).

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 dating. The ESC is not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC Guidelines and any other official rec-ommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of health care or therapeutic strategies. Health professionals areencouraged to take the ESC Guidelines fully into account when exercising their clinical judgment as well as in the determination and the implementation of preventive, diagnosticor therapeutic medical strategies. However, the ESC Guidelines do not override in any way whatsoever the individual responsibility of health professionals to make appropriateand accurate decisions in consideration of each patient’s health condition and in consultation with that patient and the patient’s caregiver where appropriate and/or necessary.Nor do the ESC Guidelines exempt health professionals from taking careful and full consideration of the relevant official updated recommendations or guidelines issued by thecompetent 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 obliga-tions. 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 and The European Society of Hypertension 2018. All rights reserved. For permissions please email: journals.permissions@oup.com.

European Heart Journal (2018) 00, 1–83 ESC GUIDELINESdoi:10.1093/eurheartj/ehy340

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Regitz-Zagrosek et al. Eur Heart J 2018

• Notions générales

• Cardiopathies

Khairy et al. JACC 2010

Épidémiologie (1)

Moons et al. Circulation 2010

Épidémiologie (2)

Marelli et al. Circulation 2014

Marelli et al Prevalence of Congenital Heart Disease 2000–2010 753

age of the most rapidly growing segment of the CHD popula-tion suggests that the next decades will witness an increas-ingly older population of patients with not only severe CHD but comorbidity that is expected to add to the disease burden.13

Improved care, decreased mortality, and improved diag-nosis over the life-span are likely contributors to the obser-vation of an increasing prevalence of CHD. We and others have found a decrease in mortality of CHD patients, as documented in Canada, the United States, and other indus-trialized nations.4,6,22 This decrease in mortality over time is likely an important contributing factor to an increasing pool of patients with prevalent CHD, especially for patients with

severe CHD. For patients with mild forms of CHD, such as aortic and mitral valve disease, the rise in prevalence from childhood to adulthood is consistent with improvement in diagnostic techniques and presentation in adulthood, with an increased likelihood of being captured with longer observa-tion periods. For example, bicuspid aortic valve is often not detected in childhood because the physical finding (a click) is subtle, and until stenosis or regurgitation develops, often not until adulthood, the diagnosis is not made. The reported rise in adults of mitral or tricuspid valve disease could be attribut-able to progressive atrioventricular valve regurgitation after endocardial cushion defect repair. Recently, we have observed a significant decrease in mortality associated with the deliv-ery of specialized care for adults with severe and other forms of CHD.23 These findings taken together provide the evidence base needed to improve quality of care for adults with CHD24 to improve outcomes.

In the Quebec CHD database, we observed a prevalence of CHD in infancy of 8.21 per 1000 from 1998 to 2005 and 8.12 per 1000 for the year 2010. Our results show that the 2010 CHD prevalence in infancy was lower than the preva-lence in childhood. The most likely explanation for this is related to ascertainment of mild CHD, which is often diag-nosed later in childhood. Therefore, it is not surprising that with up to 18 years of follow-up in children, we captured higher rates of total CHD prevalence, which included both

Figure 2. The lifetime prevalence of congenital heart disease in children and adults in Quebec, Canada, in 2010. 95%CI indicates 95% credible interval.

Figure 3. The numbers and proportions of adults and children in Quebec, Canada, with all (A) and severe (B) congenital heart disease over time in 2000, 2005, and 2010.

at INSERM - DISC on March 13, 2016http://circ.ahajournals.org/Downloaded from

Marelli et al Prevalence of Congenital Heart Disease 2000–2010 753

age of the most rapidly growing segment of the CHD popula-tion suggests that the next decades will witness an increas-ingly older population of patients with not only severe CHD but comorbidity that is expected to add to the disease burden.13

Improved care, decreased mortality, and improved diag-nosis over the life-span are likely contributors to the obser-vation of an increasing prevalence of CHD. We and others have found a decrease in mortality of CHD patients, as documented in Canada, the United States, and other indus-trialized nations.4,6,22 This decrease in mortality over time is likely an important contributing factor to an increasing pool of patients with prevalent CHD, especially for patients with

severe CHD. For patients with mild forms of CHD, such as aortic and mitral valve disease, the rise in prevalence from childhood to adulthood is consistent with improvement in diagnostic techniques and presentation in adulthood, with an increased likelihood of being captured with longer observa-tion periods. For example, bicuspid aortic valve is often not detected in childhood because the physical finding (a click) is subtle, and until stenosis or regurgitation develops, often not until adulthood, the diagnosis is not made. The reported rise in adults of mitral or tricuspid valve disease could be attribut-able to progressive atrioventricular valve regurgitation after endocardial cushion defect repair. Recently, we have observed a significant decrease in mortality associated with the deliv-ery of specialized care for adults with severe and other forms of CHD.23 These findings taken together provide the evidence base needed to improve quality of care for adults with CHD24 to improve outcomes.

In the Quebec CHD database, we observed a prevalence of CHD in infancy of 8.21 per 1000 from 1998 to 2005 and 8.12 per 1000 for the year 2010. Our results show that the 2010 CHD prevalence in infancy was lower than the preva-lence in childhood. The most likely explanation for this is related to ascertainment of mild CHD, which is often diag-nosed later in childhood. Therefore, it is not surprising that with up to 18 years of follow-up in children, we captured higher rates of total CHD prevalence, which included both

Figure 2. The lifetime prevalence of congenital heart disease in children and adults in Quebec, Canada, in 2010. 95%CI indicates 95% credible interval.

Figure 3. The numbers and proportions of adults and children in Quebec, Canada, with all (A) and severe (B) congenital heart disease over time in 2000, 2005, and 2010.

at INSERM - DISC on March 13, 2016http://circ.ahajournals.org/Downloaded from

Méconnaissance de la cardiopathie

Van Deyk et al. Am J Cardiol 2010

91 patients, âge médian 17 ans

Van Deyk et al. Am J Cardiol 2010

Perdus de vue (1)

Mackie et al. Circulation 2009

643 patients, nés en 1983, vivants à 22 ans et avec diagnostic de CHD avant l�âge de 6 ans, au Canada

13% de cardiopathies complexes, 61% de simples shunts

Facteurs prédictifs: sexe masculin, lésion peu sévère, suivi dans un centre non universitaire

Mackie et al. Circulation 2009

21% des patients avec cardiopathie complexe perdus de vue après 18 ans

Perdus de vue (2)

Quelles conséquences? (1)

Warnes. JACC 2005

Quelles conséquences? (2)

Gurvitz et al. JACC 2007

CHD

non CHD

Hospitalisations en urgence

Quelles conséquences? (3)Complications évitables

De Bono et al. Int J Cardiol 2012

Coarctation aortique

41% HTA

Soufflet et al. Am J Cardiol 2010

Fermeture spontanée

8 (4 %)

Mortalité 2 (1%)

Insuffisance valvulaire aortique

53 (26 %)

Arythmies V 1 (0.5 %)

Troubles de conduction

2 (1 %)

Endocardite infectieuse

8 (4 %)

Occlusion instrumentale

15 (7 %)

CIV pm

Oosterhof et al. Circulation 2007

Pour normaliser les volumes VD en post-opératoire: RVP quand VTDVD <160 ml/m2 ou VTSVD<82 ml/m2

Tétralogie de Fallot

Les congénitaux adultes sont-ils seulement de grands enfants?

• Changement de problèmes médicaux• Cardiopathies acquises surajoutées (ischémie

coronaire, HTA…)• Techniques d�examens différentes• Considérations sociales: emploi, assurance,

revenus, logements, prêts…• Contraception et grossesse

Transition-Principes

Kovacs et al. Prog Cardiovasc Dis 2011

• Support institutionnel

• Coordinateur de transition

• Plan de transition écrit

• Faciliter la communication directe

patient-soignant

• Education permanente

des parents et du patient

• Consultations spécifiques de transition

Recommandations européennes

Deanfield et al. Eur Heart J 2003

Recommandations américaines

Sable et al. Circulation 2011

Quelles thématiques aborder?

Sable et al. Circulation 2011

• Connaissance de la cardiopathie

• Modalités de suivi et enjeux

• Conseil génétique, risque de récidive

• Sexualité, contraception, grossesse

• Activité physique possible, limites

• Apports alimentaires

• Tabac, alcool, drogues

• Emplois possibles

• Assurances, prêts

• Support psychologique?

• Notions générales

• Cardiopathies

Mme Marine A, ddn 18/07/1981

• Tétralogie de Fallot

• Cure complète le 11 septembre 1984 (large patch d�élargissement infundibulo-pulmonaire, fermeture de CIV)

• Suivi aléatoire (asymptomatique)

• 1 grossesse sans complications en 2008

• Revient avant nouvelle grossesse…

• Se dit asymptomatique

• BDC réguliers, SS 2/6 RSG, SD court 3/6 RSG, absence d�IVD

Que lui dites-vous?

• Aucun problème!

• Nécessité d�un bilan préalable avec ETT et HolterECG

• Bilan plus complet

• Contre-indication absolue

• Cathétérisme cardiaque

Revalvulation pulmonaire chirurgicale (homogreffe) et annuloplastie tricuspide

VTDVD 234 ml/m2

VTDVD 125 ml/m2

• 1 an après : grossesse

Brickner et al. NEJM 2000