“Adults with congenital heart defects”: current challenges in medical care. Part I: the problem, structure of care, heart failure, cardiac arrhythmias

Die Kardiologie - Tập 17 Số 4 - Trang 219-233 - 2023
Harald Kaemmerer1, Stephan Baldus2, Helmut Baumgartner3, Felix Berger4, Fokko de Haan5, Stefan Frantz6, Dirk Loßnitzer7, Anselm Uebing8, Corinna Lebherz9, Michael Weyand10, Gerhard‐Paul Diller3
1Internationales Zentrum für Erwachsene mit angeborenem Herzfehler, Klinik für angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum München, Klinik an der Technischen Universität München, Lazarettstr. 36, 80636, München, Deutschland
2Medizinische Fakultät und Uniklinik Köln, Klinik III für Innere Medizin, Universität zu Köln, Kerpener Str 62, 50937, Köln, Deutschland
3Klinik für Kardiologie III: angeborene Herzfehler (EMAH) und Klappenerkrankungen, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, 48141, Münster, Deutschland
4Deutsches Herzzentrum der Charité, Klinik für Angeborene Herzfehler-Kinderkardiologie, Charité-Campus Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Deutschland
5Historisches Archiv, Deutsche Gesellschaft für Kardiologie – Herz- und Kreislaufforschung e. V., Grafenberger Allee 100, 40237, Düsseldorf, Deutschland
6Medizinische Klinik und Poliklinik I, Universitätsklinikum Würzburg, Oberdürrbacher Str. 6, 97080, Würzburg, Deutschland
7Klinik für Kardiologie, Angiologie und Pneumologie, Klinik für Kinderkardiologie / angeborene Herzfehler, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 430, 69120, Heidelberg, Deutschland
8Klinik für angeborene Herzfehler und Kinderkardiologie, Universitätsklinikum Schleswig-Holstein, Arnold-Heller-Str. 3, Haus 9, 24105, Kiel, Deutschland
9Medizinische Klinik I – Kardiologie, Angiologie, Internistische Intensivmedizin, Universitätsklinikum Aachen, Pauwelsstr. 30, 52074, Aachen, Deutschland
10Herzchirurgische Klinik, Universitätsklinikum Erlangen, Krankenhausstr. 12, 91054, Erlangen, Deutschland

Tóm tắt

Từ khóa


Tài liệu tham khảo

Osterspey A (2006) Erstellung von Leitlinien in der Deutschen Gesellschaft für Kardiologie – Herz- und Kreislaufforschung e. V. Clin Res Cardiol 95(12):692–695

Kaemmerer H, Freilinger S, Neidenbach R, Achenbach S, Andonian C, Ewert P et al (2022) Versorgung von Erwachsenen mit angeborenen Herzfehlern in Deutschland – tragende Rolle von Internisten und Hausärzten. Internist 63(1):95–102

Kaemmerer H, Andonian C, Ewert P, Freilinger S, Nagdyman N, Neidenbach R et al (2021) Managing transition—Medical follow-up care for adults with congenital heart defects. Dtsch Med Wochenschr 146(18):1192–1199

Andonian C, Beckmann J, Biber S, Ewert P, Freilinger S, Kaemmerer H et al (2018) Current research status on the psychological situation of adults with congenital heart disease. Cardiovasc Diagn Ther 8(6):799–804

Andonian C, Beckmann J, Ewert P, Freilinger S, Kaemmerer H, Oberhoffer-Fritz R et al (2020) Assessment of the psychological situation in adults with congenital heart disease. J Clin Med 9(3):779

Andonian CS, Freilinger S, Achenbach S, Ewert P, Gundlach U, Hoerer J et al (2021) ‘Well-being paradox’ revisited: a cross-sectional study of quality of life in over 4000 adults with congenital heart disease. BMJ Open 11(6):e49531

Neidenbach R et al (2021) Systematic assessment of health care perception in adults with congenital heart disease in Germany. CDT J 481–491. https://doi.org/10.21037/cdt-20-825

Deutsche Herzstiftung e. V. (2020) Deutscher Herzbericht 2019

Seidel L, Nebel K, Achenbach S, Bauer U, Ewert P, Freilinger S et al (2020) Facts about the general medical care of adults with congenital heart defects: experience of a tertiary care center. J Clin Med 9(6):1943

Deutsche Herzstiftung e. V. (2021) Leben mit angeborenen Herzfehlern im Erwachsenenalter – Ein Leitfaden

Diller GP, Orwat S, Lammers AE, Radke RM, De-Torres-Alba F, Schmidt R et al (2021) Lack of specialist care is associated with increased morbidity and mortality in adult congenital heart disease: a population-based study. Eur Heart J 42(41):4241–4248

Mackie AS, Ionescu-Ittu R, Therrien J, Pilote L, Abrahamowicz M, Marelli AJ (2009) Children and adults with congenital heart disease lost to follow-up: who and when? Circulation 120(4):302–309

Gurvitz M, Valente AM, Broberg C, Cook S, Stout K, Kay J et al (2013) Prevalence and predictors of gaps in care among adult congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial). J Am Coll Cardiol 61(21):2180–2184

Wray J, Frigiola A, Bull C (2013) Loss to specialist follow-up in congenital heart disease; out of sight, out of mind. Heart 99(7):485–490

Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M (2014) Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation 130(9):749–756

van der Linde D, Konings EE, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJ et al (2011) Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 58(21):2241–2247

Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP et al (2010) Mortality in adult congenital heart disease. Eur Heart J 31(10):1220–1229

Zomer AC, Vaartjes I, van der Velde ET, de Jong HM, Konings TC, Wagenaar LJ et al (2013) Heart failure admissions in adults with congenital heart disease; risk factors and prognosis. Int J Cardiol 168(3):2487–2493

Brida M, Lovric D, Griselli M, Riesgo Gil F, Gatzoulis MA (2022) Heart failure in adults with congenital heart disease. Int J Cardiol 357:39–45

Menachem JN, Schlendorf KH, Mazurek JA, Bichell DP, Brinkley DM, Frischhertz BP et al (2020) Advanced heart failure in adults with congenital heart disease. JACC Heart Fail 8(2):87–99

Brida M, Diller GP, Gatzoulis MA (2018) Systemic right ventricle in adults with congenital heart disease: anatomic and phenotypic spectrum and current approach to management. Circulation 137(5):508–518

Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP et al (2021) 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 42(6):563–645

Budts W, Roos-Hesselink J, Radle-Hurst T, Eicken A, McDonagh TA, Lambrinou E et al (2016) Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. Eur Heart J 37(18):1419–1427

Leczycki P, Banach M, Maciejewski M, Bielecka-Dabrowa A (2022) Heart failure risk predictions and prognostic factors in adults with congenital heart diseases. Front Cardiovasc Med 9:692815

McDonagh TA, Metra M, Adamo M, Gardner RS, Baumbach A, Bohm M et al (2021) 2021 ESC guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 42(36):3599–3726

Dodd K, Lampert BC (2018) The use and indication of Ivabradine in heart failure. Heart Fail Clin 14(4):493–500

Zengin E, Sinning C, Blaum C, Blankenberg S, Rickers C, von Kodolitsch Y et al (2021) Heart failure in adults with congenital heart disease: a narrative review. Cardiovasc Diagn Ther 11(2):529–537

Ephrem G, McCollum JC, Green-Hess D, Guglin ME, Sawada SG, Rao RA (2022) Subjective and objective impact of Angiotensin receptor-neprilysin inhibitors on systemic right ventricle patients. Heart Lung Circ 31(7):964–973

Maurer SJ, Pujol Salvador C, Schiele S, Hager A, Ewert P, Tutarel O (2020) Sacubitril/valsartan for heart failure in adults with complex congenital heart disease. Int J Cardiol 300:137–140

Packer M, Butler J, Zannad F, Filippatos G, Ferreira JP, Pocock SJ et al (2021) Effect of Empagliflozin on worsening heart failure events in patients with heart failure and preserved ejection fraction: EMPEROR-preserved trial. Circulation 144(16):1284–1294

Armstrong PW, Pieske B, Anstrom KJ, Ezekowitz J, Hernandez AF, Butler J et al (2020) Vericiguat in patients with heart failure and reduced ejection fraction. N Engl J Med 382(20):1883–1893

Gunderson EG, Lillyblad MP, Fine M, Vardeny O, Berei TJ (2019) Tolvaptan for volume management in heart failure. Pharmacotherapy 39(4):473–485

Hernandez-Madrid A, Paul T, Abrams D, Aziz PF, Blom NA, Chen J et al (2018) Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital heart disease, endorsed by HRS, PACES, APHRS, and SOLAECE. Europace 20(11):1719–1753

Boucek D, Yetman AT, Yeung E, Miyamoto SD, Stehlik J, Kfoury AG et al (2014) Survival based on patient selection for heart transplant in adults with congenital heart disease: a multi-institutional study. Int J Cardiol 172(1):e89–90

Everitt MD, Donaldson AE, Stehlik J, Kaza AK, Budge D, Alharethi R et al (2011) Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS). J Heart Lung Transplant 30(4):395–401

Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N et al (2009) Atrial arrhythmias in adults with congenital heart disease. Circulation 120(17):1679–1686

Khairy P, Harris L, Landzberg MJ, Fernandes SM, Barlow A, Mercier LA et al (2008) Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study. Circ Arrhythm Electrophysiol 1(4):250–257

Wasmer K, Eckardt L, Baumgartner H, Kobe J (2021) Therapy of supraventricular and ventricular arrhythmias in adults with congenital heart disease-narrative review. Cardiovasc Diagn Ther 11(2):550–562

Labombarda F, Hamilton R, Shohoudi A, Aboulhosn J, Broberg CS, Chaix MA et al (2017) Increasing prevalence of atrial fibrillation and permanent atrial arrhythmias in congenital heart disease. J Am Coll Cardiol 70(7):857–865

Wasmer K, Kobe J, Dechering DG, Bittner A, Monnig G, Milberg P et al (2013) Isthmus-dependent right atrial flutter as the leading cause of atrial tachycardias after surgical atrial septal defect repair. Int J Cardiol 168(3):2447–2452

Waldmann V, Khairy P (2021) Pathophysiology, risk factors, and management of atrial fibrillation in adult congenital heart disease. Card Electrophysiol Clin 13(1):191–199

de Groot NM, Zeppenfeld K, Wijffels MC, Chan WK, Blom NA, Van der Wall EE et al (2006) Ablation of focal atrial arrhythmia in patients with congenital heart defects after surgery: role of circumscribed areas with heterogeneous conduction. Heart Rhythm 3(5):526–535

Iturralde P, Nava S, Salica G, Medeiros A, Marquez MF, Colin L et al (2006) Electrocardiographic characteristics of patients with Ebstein’s anomaly before and after ablation of an accessory atrioventricular pathway. J Cardiovasc Electrophysiol 17(12):1332–1336

Kapel GF, Sacher F, Dekkers OM, Watanabe M, Blom NA, Thambo JB et al (2017) Arrhythmogenic anatomical isthmuses identified by electroanatomical mapping are the substrate for ventricular tachycardia in repaired Tetralogy of Fallot. Eur Heart J 38(4):268–276

Kobe J, Willy K, Eckardt L, Baumgartner H, Wasmer K (2021) Narrative review of: risk stratification and implantable cardioverter-defibrillator therapy in adults with congenital heart disease. Cardiovasc Diagn Ther 11(2):538–549

Khairy P, Van Hare GF, Balaji S, Berul CI, Cecchin F, Cohen MI et al (2014) PACES/HRS Expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed 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). Heart Rhythm 11(10):e102–65

Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E et al (2016) Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management. Eur J Pediatr 175(9):1235–1248

Huhta JC, Maloney JD, Ritter DG, Ilstrup DM, Feldt RH (1983) Complete atrioventricular block in patients with atrioventricular discordance. Circulation 67(6):1374–1377

Liberman L, Silver ES, Chai PJ, Anderson BR (2016) Incidence and characteristics of heart block after heart surgery in pediatric patients: A multicenter study. J Thorac Cardiovasc Surg 152(1):197–202

Lin A, Mahle WT, Frias PA, Fischbach PS, Kogon BE, Kanter KR et al (2010) Early and delayed atrioventricular conduction block after routine surgery for congenital heart disease. J Thorac Cardiovasc Surg 140(1):158–160

Batra AS, Wells WJ, Hinoki KW, Stanton RA, Silka MJ (2003) Late recovery of atrioventricular conduction after pacemaker implantation for complete heart block associated with surgery for congenital heart disease. J Thorac Cardiovasc Surg 125(6):1291–1293

Squarcia U, Merideth J, McGoon DC, Weidman WH (1971) Prognosis of transient atrioventricular conduction disturbances complicating open heart surgery for congenital heart defects. Am J Cardiol 28(6):648–652

Ghonim S, Gatzoulis MA, Ernst S, Li W, Moon JC, Smith GC et al (2022) Predicting survival in repaired tetralogy of Fallot: a lesion-specific and personalized approach. JACC Cardiovasc Imaging 15(2):257–268

Hindricks G, Potpara T, Dagres N, Arbelo E, Bax JJ, Blomstrom-Lundqvist C et al (2021) 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS): The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology (ESC) Developed with the special contribution of the European Heart Rhythm Association (EHRA) of the ESC. Eur Heart J 42(5):373–498

Yang H, Bouma BJ, Dimopoulos K, Khairy P, Ladouceur M, Niwa K et al (2020) Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study. Int J Cardiol 299:123–130

Freisinger E, Gerss J, Makowski L, Marschall U, Reinecke H, Baumgartner H et al (2020) Current use and safety of novel oral anticoagulants in adults with congenital heart disease: results of a nationwide analysis including more than 44 000 patients. Eur Heart J 41(43):4168–4177

Koyak Z, Kroon B, de Groot JR, Wagenaar LJ, van Dijk AP, Mulder BA et al (2013) Efficacy of antiarrhythmic drugs in adults with congenital heart disease and supraventricular tachycardias. Am J Cardiol 112(9):1461–1467

Fish FA, Gillette PC, Benson DW Jr. (1991) Proarrhythmia, cardiac arrest and death in young patients receiving encainide and flecainide. The Pediatric Electrophysiology Group. J Am Coll Cardiol 18(2):356–365

Konemann H, Frommeyer G, Zeppenfeld K, Eckardt L (2023) The new ESC guidelines on the management of ventricular tachyarrhythmias: Implications for daily practice. Herz 48(1):3–14

van Zyl M, Kapa S, Padmanabhan D, Chen FC, Mulpuru SK, Packer DL et al (2016) Mechanism and outcomes of catheter ablation for ventricular tachycardia in adults with repaired congenital heart disease. Heart Rhythm 13(7):1449–1454

Moore JP, Seki A, Shannon KM, Mandapati R, Tung R, Fishbein MC (2013) Characterization of anatomic ventricular tachycardia isthmus pathology after surgical repair of tetralogy of Fallot. Circ Arrhythm Electrophysiol 6(5):905–911

Zeppenfeld K, Tfelt-Hansen J, de Riva M, Winkel BG, Behr ER, Blom NA et al (2022) 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 43(40):3997–4126

Bertels RA, Harteveld LM, Filippini LH, Clur SA, Blom NA (2017) Left ventricular dysfunction is associated with frequent premature ventricular complexes and asymptomatic ventricular tachycardia in children. Europace 19(4):617–621

Janousek J, van Geldorp IE, Krupickova S, Rosenthal E, Nugent K, Tomaske M et al (2013) Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study. Circulation 127(5):613–623

Glikson M, Nielsen JC, Kronborg MB, Michowitz Y, Auricchio A, Barbash IM et al (2021) 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J 42(35):3427–3520

Opic P, Yap SC, Van Kranenburg M, Van Dijk AP, Budts W, Vliegen HW et al (2013) Atrial-based pacing has no benefit over ventricular pacing in preventing atrial arrhythmias in adults with congenital heart disease. Europace 15(12):1757–1762

Krongrad E (1978) Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects. Circulation 57(5):867–870

Koyak Z, Harris L, de Groot JR, Silversides CK, Oechslin EN, Bouma BJ et al (2012) Sudden cardiac death in adult congenital heart disease. Circulation 126(16):1944–1954

Kella DK, Merchant FM, Veledar E, Book W, Lloyd MS (2014) Lesion-specific differences for implantable cardioverter defibrillator therapies in adults with congenital heart disease. Pacing Clin Electrophysiol 37(11):1492–1498

Santharam S, Hudsmith L, Thorne S, Clift P, Marshall H, De Bono J (2017) Long-term follow-up of implantable cardioverter-defibrillators in adult congenital heart disease patients: indications and outcomes. Europace 19(3):407–413

Frommeyer G, Feder S, Bettin M, Debus V, Kobe J, Reinke F et al (2018) Long-term single-center experience of defibrillator therapy in children and adolescents. Int J Cardiol 271:105–108

Vehmeijer JT, Brouwer TF, Limpens J, Knops RE, Bouma BJ, Mulder BJ et al (2016) Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis. Eur Heart J 37(18):1439–1448

Silvetti MS, Kornyei L, Kwiatkowska J, Tamburri I, Saputo FA, Pazzano V et al (2019) SICD registry in European paediatric and adult patients with congenital heart defects: preliminary results of the SIDECAR project. Eur Heart J 40:3119