Introduction to the focused series on “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V”

Introduction to the focused series on “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V”

Medical care for patients with congenital heart disease (CHD) has currently reached a high, unprecedented level (1). Children with CHD now reach adulthood in more than 95% of industrialized countries, and the number of adults with congenital heart defects (ACHD) is estimated at 50 million worldwide and will steadily rise in the next decades (2,3).

Most CHDs are repaired rather than completely corrected, anatomic and functional residua and sequelae often exist. Cardiac defect-specific late complications such as heart failure, arrhythmias, pulmonary vascular disease, aortopathies, infective endocarditis, or concomitant acquired organ disease affect the quality of life (QOL), performance, work capacity, and mental health of ACHD with advancing age (4,5). If treated inappropriately, further development of heart failure, arrhythmias, and possibly premature death will occur. Therefore, most patients with CHD will require life-long follow-up from a multidisciplinary team as a major determinant. This requires that all treating physicians are up to date with the latest knowledge and also continuously educate themselves in this area (6).

At a new era of the planet, with an immense number of refugees and immigrants due to regional wars and economic inequality, we need to consider bringing the scientific level of all international congenital cardiac heart centers to a certain advanced level, regardless of our own countries’ borders.

We have always to keep in mind that a patient with CHD that was incompletely repaired in a remote country may turn out to be a candidate that can still be treated medically and with cardiac surgery in a developed country.

In this regard, the need of networking and international cooperation, as well as remote teaching tools like journals and publications in congenital cardiac disease and congenital heart surgery, appear to be a very important international contribution to public healthcare.

In this 5th volume of a series on ACHD, international authors again present new research findings and communicate relevant aspects of ACHD care. This volume includes 19 excellent papers; 15 original and 4 review papers. Recent data on cardiac failure, pulmonary hypertension, aortopathies, vascular aging, QOL and psychological issues, health care status, transposition of the great arteries after atrial switch procedures, and Ebstein’s anomaly are presented. Also, interesting findings on bicuspid aortic valve, Fabry disease, left ventricular (LV) noncompaction and Fontan procedure are provided.

We hope that this issue of the CDT journal, which reports on important, new, and also practice-relevant aspects of ACHD care, will help expand knowledge about ACHD for young colleagues and further develop ACHD care. We would like to thank the CDT journal and especially Prof. Paul Schoenhagen, editor in chief, for their dedication to the field of ACHD and for giving the topic of “Adult Congenital Heart Disease”.


Funding: None.


Provenance and Peer Review: This article was commissioned by the editorial office, Cardiovascular Diagnosis and Therapy for the series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V”. The article did not undergo external peer review.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V” was commissioned by the editorial office without any funding or sponsorship. KN, HK and YVK served as the unpaid Guest Editors of the series. HK reports sponsorship/honoraria/meeting or travel support received from Actelion/Janssen, Bristol-Myers Squibb, research grant/support from patient organisation: Deutsche Herzstiftung and Herzkind e.V, and participates on steering board for COMPERA International Steering Board. The authors have no other conflicts of interests to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See:


  1. Hoffman JIe. The global burden of congenital heart disease. Cardiovasc J Afr 2013;24:141-5. [Crossref] [PubMed]
  2. Henning RJ. Diagnosis and treatment of adults with congenital heart disease. Future Cardiol 2020;16:317-42. [Crossref] [PubMed]
  3. Benziger CP, Stout K, Zaragoza-Macias E, et al. Projected growth of the adult congenital heart disease population in the United States to 2050: an integrative systems modeling approach. Popul Health Metr 2015;13:29. [Crossref] [PubMed]
  4. Neidenbach R, Niwa K, Oto O, et al. 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. Cardiovasc Diagn Ther 2018;8:705-15. [Crossref] [PubMed]
  5. Neidenbach R, Niwa K, Oto O, et al. Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy. Cardiovasc Diagn Ther 2018;8:716-24. [Crossref] [PubMed]
  6. 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. [Crossref] [PubMed]
Koichiro Niwa
Yskert von Kodolitsch
Öztekin Oto
Harald Kaemmerer

Koichiro Niwa, MD, PhD

Executive Advisor, Cardiovascular Center, St. Luke’s International Hospital, Tokyo, Japan.(Email:

Yskert von Kodolitsch, MD, MBA

Professor of Internal Medicine/Cardiology, Klinik für Gefäßmedizin, Universitäres Herz- und Gefäßzentrum Hamburg, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. (Email:

Öztekin Oto, MD, FESC, FACC

Professor of Cardiovascular Surgery, Department of Cardiovascular Surgery, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey. (Email:

Harald Kaemmerer, MD, VMD

Professor of Internal Medicine/Cardiology, International Center for Adult Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany. (Email:

Keywords: Congenital heart disease (CHD); follow-up; sequelae; residua; complications

Submitted Mar 10, 2023. Accepted for publication Mar 30, 2023. Published online Apr 06, 2023.

doi: 10.21037/cdt-2023-1

Cite this article as: Niwa K, von Kodolitsch Y, Oto Ö, Kaemmerer H. Introduction to the focused series on “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part V”. Cardiovasc Diagn Ther 2023;13(2):323-325. doi: 10.21037/cdt-2023-1

Download Citation