Cardiac Arrhythmias in Adults with Congenital Heart Disease: An Observational Study over 15 Years

All articles of this category (opens in new window) Background: Cardiac arrhythmias have a decisive impact on mortality and morbidity in adults with congenital heart disease (ACHD). The aim of this study was to determine the arrhythmia burden and therapeutic interventions over the long-term course in patients with congenital heart defects (CHD). Methods: Between 2003 and 2004, 364 adults with a surgically treated CHD were enrolled in the Göttingen “Lebenschancen” (Life Chances; LC1) Study. At follow-up 15 years later, a total of 249/364 patients with a median age of 38 (IQR: 33–47) years were re-evaluated. The prevalence of arrhythmias, the frequency of electrophysiological studies and catheter-based ablations, as well as the presence of cardiac implantable electronic devices (CIED), were analyzed within this 15-year interval. Results: Overall, atrial and ventricular tachy-arrhythmias occurred in 16.1% of all patients. Atrial tachy-arrhythmias (AT) were most common (14.1%), while ventricular tachy-arrhythmias (VT) occurred in 8.4%. Bradyarrhythmias were less common (Sick sinus syndrome and atrioventricular block; 9.2%). Arrhythmias were most frequent in patients with Fontan circulation (44.4%; AT only), followed by patients with d-transposition of the great arteries without arterial switch (dTGA; 31.6%) and patients with tetralogy of Fallot (TOF; 23.5%). In dTGA and TOF, AT were more frequent than VT (26.3% vs. 15.8% in dTGA; 25.5% vs. 9.8% in TOF). The highest prevalence of VT was observed in patients with aortic valve disease (18.5%). Electrophysiological studies (EPS) were performed in 15.3% of patients (most often in Fontan patients: 44%; followed by D-TGA: 36.8%) within this 15-year interval. Catheter ablation was performed in 10.8% of all patients. CIEDs were implanted in 10.4% of patients during the observation period. By the end of the study in 2019, 19.3% of all patients had a device (pacemakers 7.6%, implantable cardioverter-defibrillators (ICDs) 11.6%), including those patients who got a device before LC1. ICDs were most frequently used in patients with D-TGA (42.1%), followed by TOF (23.5%); pacemakers were also most frequent in D-TGA (26.3%), followed by Fontan (11.1%). Conclusion: Cardiac arrhythmias were an important complication in our study cohort, with the highest burden in patients with Fontan circulation, dTGA, and TOF, necessitating specific interventional therapy by means of EPS/ablation or implantation of rhythmologically effective devices. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany