Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia

Rhoia Neidenbach¹, Koichiro Niwa², Oeztekin Oto³, Erwin Oechslin⁴, Jamil Aboulhosn⁵, David Celermajer⁶, Joerg Schelling⁷, Lars Pieper⁸, Linda Sanftenberg⁷, Renate Oberhoffer^{1

9}, Fokko de Haan¹⁰, Michael Weyand¹¹, Stephan Achenbach¹², Christian Schlensak¹³, Dirk Lossnitzer¹⁴, Nicole Nagdyman¹, Yskert von Kodolitsch¹⁵, Hans-Carlo Kallfelz¹⁶, David Pittrow¹⁷, Ulrike M M Bauer¹⁸, Peter Ewert¹, Thomas Meinertz¹⁵, Harald Kaemmerer¹

Affiliations

¹ Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany.
² Department of Cardiology, Cardiovascular Center, St Luke's International Hospital, Tokyo, Japan.
³ Dokuz Eylul University Hospital air Esref Cad, Izmir, Turkey.
⁴ Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, Toronto General Hospital, and University of Toronto, Toronto, Ontario, Canada.
⁵ Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, USA.
⁶ Central Clinical School Heart Research Institute C39 - Royal Prince Alfred Hospital, The University of Sydney, NSW 2006, Australia.
⁷ Institute of General Practice and Family Medicine, University Hospital of Ludwig-Maximilians-University Munich, Munich, Germany.
⁸ Chair of Behavioral Epidemiology, Institute for Clinical Psychology und Psychotherapy, Technical University Dresden, Dresden, Germany.
⁹ Preventive Pediatrics, Technical University Munich, Munich, Germany.
¹⁰ Cardiological Clinic Solingen, Solingen, Germany.
¹¹ Department of Cardiac Surgery, University of Erlangen, Erlangen, Germany.
¹² Department of Cardiology, University of Erlangen, Erlangen, Germany.
¹³ Clinic for Thorax-, Heart- and Vessel Surgery, German Heart Competence Center, Tübingen, Germany.
¹⁴ Medical Clinic I, University Mannheim, Mannheim, Germany.
¹⁵ University Heart Center Hamburg, University Clinic Hamburg-Eppendorf, Hamburg, Germany.
¹⁶ Clinic for Pediatric Cardiology und Intensive medicine, Medical School Hannover, Hannover, Germany.
¹⁷ Institute for Clinical Pharmacology, Medical Faculty, Technical University Carl Gustav Carus, Dresden, Germany.
¹⁸ Competence Network Congenital Heart Defects, Berlin, Germany.

PMID: 30740318
PMCID: PMC6331379
DOI: 10.21037/cdt.2018.10.15

Review

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia

Rhoia Neidenbach et al. Cardiovasc Diagn Ther. 2018 Dec.

. 2018 Dec;8(6):705-715.

doi: 10.21037/cdt.2018.10.15.

Authors

Affiliations

¹ Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany.
² Department of Cardiology, Cardiovascular Center, St Luke's International Hospital, Tokyo, Japan.
³ Dokuz Eylul University Hospital air Esref Cad, Izmir, Turkey.
⁴ Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network, Toronto General Hospital, and University of Toronto, Toronto, Ontario, Canada.
⁵ Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, USA.
⁶ Central Clinical School Heart Research Institute C39 - Royal Prince Alfred Hospital, The University of Sydney, NSW 2006, Australia.
⁷ Institute of General Practice and Family Medicine, University Hospital of Ludwig-Maximilians-University Munich, Munich, Germany.
⁸ Chair of Behavioral Epidemiology, Institute for Clinical Psychology und Psychotherapy, Technical University Dresden, Dresden, Germany.
⁹ Preventive Pediatrics, Technical University Munich, Munich, Germany.
¹⁰ Cardiological Clinic Solingen, Solingen, Germany.
¹¹ Department of Cardiac Surgery, University of Erlangen, Erlangen, Germany.
¹² Department of Cardiology, University of Erlangen, Erlangen, Germany.
¹³ Clinic for Thorax-, Heart- and Vessel Surgery, German Heart Competence Center, Tübingen, Germany.
¹⁴ Medical Clinic I, University Mannheim, Mannheim, Germany.
¹⁵ University Heart Center Hamburg, University Clinic Hamburg-Eppendorf, Hamburg, Germany.
¹⁶ Clinic for Pediatric Cardiology und Intensive medicine, Medical School Hannover, Hannover, Germany.
¹⁷ Institute for Clinical Pharmacology, Medical Faculty, Technical University Carl Gustav Carus, Dresden, Germany.
¹⁸ Competence Network Congenital Heart Defects, Berlin, Germany.

PMID: 30740318
PMCID: PMC6331379
DOI: 10.21037/cdt.2018.10.15

Abstract

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.

Keywords: Congenital heart defect (CHD); endocarditis; heart failure; primary health care; pulmonary hypertension.

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Conflict of interest statement

Conflicts of Interest: R Neidenbach received research grants (“Unrestricted educational grant”) from Actelion Pharmaceuticals Deutschland GmbH and from the German Heart Foundation (“Deutsche Herzstiftung e.V.) and the patient organization “Herzkind e. V.”. H Kaemmerer received fees and/or travel expenses for consulting activities and/or lectures from the following companies within the last 3 years: Actelion, Pfizer, Bayer-Healthcare, Bristol-Myers Squibb. D Pittrow has received speaker fees or honoraria for consultations from Actelion, Bayer, Genzyme, Boehringer Ingelheim, Novartis, MSD, and Pfizer. E Oechslin currently holds the “Bitove Family Professorship for Adult Congenital Heart Disease”. Other authors have no conflicts of interest to declare.

Figures

**Figure 1**
Annual births of children with congenital heart disease. (A) Total annual births of children with CHD by continent. The incidence or birth prevalence of CHD largely depends on the study design (3); (B) number of children born with CHD per million population. The incidence or birth prevalence of CHD largely depends on the study design (3). CHD, congenital heart disease. From Hoffman (6). Reprinted with permission.

**Figure 2**
Estimated clinical relevance of CHD in the next years from the German National Register for Congenital Heart Defects (12). CHD, congenital heart disease. Reprinted with permission. Copyright by: Nationales Register für angeborene Herzfehler, 2016 (12).

**Figure 3**
Medical treatment for heart failure related to intrinsic myocardial dysfunction. From Budts *et al.* (30). Adapted with permission.

**Figure 4**
Management algorithms for the care of adults with congenital heart disease.

See this image and copyright information in PMC

References

1. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018. AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2018. [Epub ahead of print]. - PubMed
1. Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation 2017;136:e348-92. 10.1161/CIR.0000000000000535 - DOI - PubMed
1. Neidenbach RC, Lummert E, Vigl M, et al. Non-cardiac comorbidities in adults with inherited and congenital heart disease: report from a single center experience of more than 800 consecutive patients. Cardiovasc Diagn Ther 2018;8:423-31. 10.21037/cdt.2018.03.11 - DOI - PMC - PubMed
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Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia

Affiliations

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

LinkOut - more resources

Full Text Sources