Sudden cardiac death in adults with congenital heart disease

Abstract

Sudden cardiac death is one of the leading causes of death in patients with congenital heart disease, especially in patients with repaired cyanotic and left heart obstructive lesions. While the overall annual incidence of sudden cardiac death is relatively low, estimated at 0.09% per year, this nonetheless represents a many-fold increase over that of comparable age-matched control populations. The most frequent cause of sudden cardiac death is believed to be arrhythmic, usually ventricular arrhythmia. Most studies investigating risk factors for ventricular arrhythmia and/or sudden cardiac death have focused on patients with repaired tetralogy of Fallot and patients with Mustard/Senning repair for complete transposition of the great arteries. Despite a multitude of risk factors, their predictive value for the occurrence of sudden cardiac death is relatively low. Current experience with implantable cardioverter defibrillators in this patient population is limited to observational studies and the selection of patients for prophylactic implantable cardioverter defibrillator implantation is impeded both by the absence of randomized trials and weak predictors. Catheter ablation of ventricular tachycardia has emerged as a promising therapy for abolishing or reducing the burden of arrhythmia but experience is still limited and the impact on long-term outcome uncertain. Future studies will have to focus on improving risk stratification of patients with congenital heart disease.

Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS)

“…Numerous studies have documented a high incidence of arrhythmias and sudden cardiac death in adults with CHD, suggesting that many would indeed benefit from ICD therapy. [15][16][17] Documentation of a high rate of appropriate ICD shocks in adults with CHD who receive an ICD for primary prevention of sudden cardiac death due to ventricular dysfunction further supports this argument. 18 Despite such data, ICD therapy is not routinely undertaken for primary prevention.…”

Sudden Cardiac Death in Adult Congenital Heart Disease

“…When applied to adults with CHD in the United States and Canada, Ϸ765 and 90 SCD events will occur annually, respectively. 10,11 Additionally, because the majority of infants born with CHD will now survive into adulthood, the prevalence of SCD will steadily increase. Adults with repaired tetralogy of Fallot (TOF) and/or Mustard or Senning repair for complete transposition of the great arteries (TGA) have been studied previously, and several potential risk factors for SCD have been identified.…”

A Novel GATA4 Loss-of-Function Mutation Associated With Congenital Ventricular Septal Defect

“…Regardless of other potential conditions that accompany VSD, isolated moderate-to-large VSD with persistent left-to-right shunting of blood may give rise to cardiac enlargement, ventricular dysfunction, pulmonary hypertension, Eisenmenger's syndrome, delayed fetal brain development, arrhythmias, and even sudden cardiac death in the absence of surgical or catheter-based repair [20,37,39,42,44]. Despite the high prevalence of VSD and its significant association with increased morbidity and mortality as well as poor quality of life, the etiology of VSD remains largely unclear [7,16,28,40].…”