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.
to prospectively study predicted SCD risk using PREVENTION-ACHD and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF)rates in ACHD patients.
The PREVENTION-ACHD risk-score assigns one point each to: coronary artery disease, NYHA class II/III heart failure, supraventricular tachycardia, systemic ejection fraction <40%, subpulmonary ejection fraction <40%, QRS duration ≥120 msec, QT dispersion ≥70 msec. SCD-risk was calculated for each patient. An annual predicted risk ≥3% constituted high-risk. The primary outcome was SCD or VT/VF after 2 years. The secondary outcome was SCD.
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<0.001). The risk-score's sensitivity was 0.5, specificity 0.93, resulting in a C-statistic of 0.75 (95%CI 0.57-0.90). The HR for SCD was 12.4 (95%CI 1.8-88.1, p=0.01); sensitivity and specificity were 0.5 and 0.92, and the C-statistic 0.81 (95%CI 0.67-0.95).
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.