Identification of patients at risk of sudden cardiac death in congenital heart disease. The PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease: PREVENTION-ACHD

Background Sudden cardiac death (SCD) is the main preventable cause of death in adult congenital heart disease (ACHD) patients. Since robust risk stratification methods are lacking, we developed a risk score to predict SCD in ACHD patients: PREVENTION-ACHD. Objective to prospectively study predicted SCD risk using PREVENTION-ACHD and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF)rates in ACHD patients. Methods The PREVENTION-ACHD risk-score assigns one point each to: coronary artery disease, NYHA class II/III heart failure, supraventricular tachycardia, systemic ejection fraction Results 783 consecutive ACHD patients (31% left-sided lesions, 18% tetralogy of Fallot, 14% closed ASD), median age 36 years and 52% male, were included. PREVENTION-ACHD identified 58 high-risk patients. Eight patients (four at high-risk) experienced the primary outcome. The Kaplan-Meier estimates were 7% (95%CI 0.1-13.3%) in the high-risk and 0.6% (95%CI 0-1.1%) in the low-risk group, HR 12.5 (95%CI 3.1-50.9, p Conclusion The PREVENTION-ACHD risk-score provides greater accuracy in SCD or VT/VF risk stratification compared to current guideline indications and identifies ACHD patients who may benefit from preventive ICD implantation.