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

Abstract

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

“…Recorded data that could have an impact on morbidity and mortality are either completely missed or remain concealed for extended periods. With high rates of device‐related complications, inappropriate therapies, and atrial arrhythmias, these events could be detected earlier and appropriate measures undertaken to reduce the number of shocks and spare the device battery . In our cohort, a significant number of patients was diagnosed with atrial fibrillation and flutter before an outpatient visit, which lead to an earlier change in medication.…”

“…ICD implantation occurred several years after the repair of congenital heart defects. Surgical scars are important substrates for ventricular arrhythmias, but the risk of SCD will only be perceived long after surgery. This emphasizes the importance of the long‐term follow‐up of these patients.…”

“…The class II indications are extrapolated from studies in patients with acquired cardiac conditions or derived from retrospective data or expert opinions. They rely on the presence of an indeterminate number of risk factors, have no defined cutoff values for left and right ventricular dysfunction, and, apart from tetralogy of Fallot, are not disease‐specific …”

“…Tailored antiarrhythmic drug therapy is associated with longer times to first shock and reduced number of shocks . Moreover, it has been suggested for patients with tetralogy of Fallot that prophylactic ablation of potential arrhythmogenic isthmuses could prevent primary prevention ICD implantation . This approach has yet to be tested in a randomized prospective study.…”

“…Secondary prevention included patients who received an ICD after suffering a life‐threatening ventricular arrhythmia, that is, sustained symptomatic VT or VF. All other patients were classified as primary prevention and were divided into class I and II indications, according to the most recent guidelines . Cardiac devices were implanted at the discretion of the attending physicians.…”