https://doi.org/10.1007/s00392-025-02737-x
1Universitätsklinikum Erlangen Herzchirurgische Klinik Erlangen, Deutschland; 2Deutsches Herzzentrum München Klinik für angeborene Herzfehler und Kinderkardiologie München, Deutschland; 3Universitätsklinikum Erlangen Medizinische Klinik 2 Erlangen, Deutschland; 4Deutsches Herzzentrum München Klinik für Chirurgie angeborener Herzfehler und Kinderherzchirurgie München, Deutschland; 5Herzzentrum der Universität zu Köln Klinik III für Innere Medizin Köln, Deutschland; 6Deutsches Herzzentrum München Institut für Laboratoriumsmedizin München, Deutschland; 7Universitätsklinikum Tübingen Pädiatrische Kardiologie Tübingen, Deutschland; 8Technische Universität Braunschweig Biostatistik Braunschweig, Deutschland; 9Universität Wien Institut für Sport- und Bewegungswissenschaft Wien, Österreich
Background:
Advances in diagnosis, treatment, and long-term care have led to a growing population of adults with congenital heart disease (ACHD). Although their life expectancy continues to rise, their clinical needs remain insufficiently characterized. Particularly cardiac and non-cardiac comorbidities emerge as a challenge and emerging data suggest a phenomenon of accelerated biological aging, with earlier onset of both cardiovascular and systemic comorbidities compared to the general population. However, data on older and even geriatric ACHD remain limited.
Objectives:
This study aimed to evaluate the clinical characteristics and burden of ACHD aged ≥50 years, with particular attention to functional status, cardiovascular and non-cardiovascular comorbidities, sex-specific differences, and therapeutic patterns.
Methods:
The PATHFINDER-CHD-Registry is a prospective, observational, web-based multicenter registry that includes ACHD and manifest HF, a history of HF, or a high risk for the development of HF. It documents the pathoanatomical diagnosis, clinical relevant data, including cardiac surgery, interventional treatments, cardiac and non-cardiac comorbidities, and medical treatments.
Results:
In this cross-sectional, multi-center study, 297 ACHD aged 50 years or older were assessed. Most patients had acyanotic CHD (n = 183; 62 %), 114 (38 %) primarily cyanotic CHD. The most prevalent congenital diagnoses were Tetralogy of Fallot (21%), Ebstein’s anomaly (12%), and aortic coarctation (11%). 37 (12%) had a morphologic right systemic ventricle and 14 (4.7%) a univentricular heart A total of 21% of patients had manifest heart failure; 44% were classified as ACC/AHA stage B and 51% as stage C. Nevertheless, 77% of patients were in Perloff functional class I/II. Cardiovascular comorbidities were common, including aortopathies (55%), systemic arterial hypertension (37%), and arrhythmias (33%). Non-cardiac comorbidities such as thyroid dysfunction (25%), renal impairment (18%), and neurological disorders (13%) were frequently observed. Women had higher rates of arrhythmia and endocrine dysfunction, while men more often exhibited aortopathies and neurological conditions. Despite the high burden of heart failure, contemporary therapies such as SGLT2 inhibitors and ARNIs were used in only 17% and 8.4% of patients, respectively.
Conclusions:
Older adults with ACHD represent a clinically complex and heterogeneous population with significant cardiovascular and systemic comorbidities. These findings support the concept of accelerated biological aging and highlight the need for individualized, interdisciplinary care models tailored to the specific vulnerabilities of this population to improve outcomes and quality of life in this expanding and vulnerable group.