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

Abstract

Objective Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardioverter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs. Design Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected. Results Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT) or ventricular fibrillation (VF). The rate of inappropriate therapies and device‐related complications was 33.3%. Secondary prevention and primary prevention patients with class I indications for ICD had more appropriate therapies than complication, but this relationship was reversed for patients with class II indications. Remote monitoring played an important role in diagnosing new atrial arrhythmias before scheduled visits in 46.2% of patients, leading to a change in medication. VT/VF episodes were associated with a composite of death, cardiac transplantation, and hospital admission (OR 13.0; 95% CI: 2.1‐81.5). Conclusion ICDs are not only useful in preventing SCD, but also have a major role in diagnosing atrial tachyarrhythmias ahead of scheduled visits. Although improvements in ICD technology might reduce complications and inappropriate therapies, adequate selection of candidates for primary prevention still remains difficult because of the lack of clear indications.

Medical Therapy for Heart Failure in Adult Congenital Heart Disease Patients

“… 85 ICDs may also help with remote monitoring of new atrial arrhythmias, which could lead to initiation and/or change of antiarrhythmic drug therapy and anticoagulation. 86 Method of implantation via transvenous or epicardial lead placement is dictated by the underlying congenital anatomy and/or surgical repair.…”

Handling of Ventricular Fibrillation in the Emergency Setting

“…The most common factors that may contribute to the development of VF are acquired risk factors, e.g., ischemic heart disease, cardiomyopathies, electrolyte imbalances, certain drug therapies affecting myocardial repolarization, and less frequently inherited disorders, e.g., ion channel abnormalities (channelopathies), congenital valvular diseases, and coronary artery anomalies. (Table 1) (Garson and McNamara, 1985;Jouven et al, 1999;Thompson et al, 2000;Frothingham, 2001;Haddad and Anderson, 2002;Taylor, 2003;Yap and Camm, 2003;Vieweg and Wood, 2004;Owens and Ambrose, 2005;Haissaguerre et al, 2008;Priori et al, 2015;Aiba, 2019;Coutinho Cruz et al, 2019).…”