TY - JOUR
T1 - Identification of patients at risk of sudden cardiac death in congenital heart disease
T2 - The PRospEctiVE study on implaNTable cardlOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD)
AU - Vehmeijer, Jim T.
AU - Koyak, Zeliha
AU - Leerink, Jan M.
AU - Zwinderman, Aeilko H.
AU - Harris, Louise
AU - Peinado, Rafael
AU - Oechslin, Erwin N.
AU - Robbers-Visser, Dani Elle
AU - Groenink, Maarten
AU - Boekholdt, S. Matthijs
AU - De Winter, Robbert J.
AU - Oliver, Jose M.
AU - Bouma, Berto J.
AU - Budts, Werner
AU - Van Gelder, Isabelle C.
AU - Mulder, Barbara J. M.
AU - de Groot, Joris R.
PY - 2021/5
Y1 - 2021/5
N2 - BACKGROUND Sudden cardiac death (SCD) is the main preventable cause of death in patients with adult congenital heart disease (ACHD). Since robust risk stratification methods are lacking, we developed a risk score model to predict SCD in patients with ACHD: the PRospEctiVE study on implaNTable cardlOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD) risk score model.OBJECTIVE The purpose of this study was to prospectively study predicted SCD risk using the PREVENTION-ACHD risk score model and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF) rates in patients with ACHD.METHODS The PREVENTION-ACHD risk score model assigns 1 point each to coronary artery disease, New York Heart Association class II/III heart failure, supraventricular tachycardia, systemic ejection fraction < 40%, subpulmonary ejection fraction < 40%, QRS duration >= 120 ms, and QT dispersion >= 70 ms. SCD risk was calculated for each patient. An annual predicted risk of >= 3% constituted high risk. The primary outcome was SCD or VT/VF after 2 years. The secondary outcome was SCD.RESULTS The study included 783 consecutive patients with ACHD (n=239 (31%) left-sided lesions; n=138 (18%) tetralogy of Fallot; n=108 (14%) dosed atrial septal defect; median age 36 years; interquartile range 28-47 years; n=401 (51%) men). The PREVENTION-ACHD risk score modelidentified 58 high-risk patients. Eight patients (4 at high risk) experienced the primary outcome. The Kaplan-Meier estimates were 7% (95% confidence interval [CI] 0.1%-13.3%) in the high-risk group and 0.6% (95% CI 0.0%-1.1%) in the low-risk group (hazard ratio 12.5; 95% CI 3.1-50.9; P < .001). The risk score model's sensitivity was 0.5 and specificity 93, resulting in a C-statistic of 0.75 (95% CI 0.57-0.90). The hazard ratio for SCD was 12.4 (95% CI 1.8-88.1) (P = .01); the sensitivity and specificity were 0.5 and 0.92, and the C-statistic was 0.81 (95% CI 0.67-0.95).CONCLUSION The PREVENTION-ACHD risk score model provides greater accuracy in SCD or VT/VF risk stratification as compared with current guideline indications and identifies patients with ACHD who may benefit from preventive implantable cardioverterdefibrillator implantation.
AB - BACKGROUND Sudden cardiac death (SCD) is the main preventable cause of death in patients with adult congenital heart disease (ACHD). Since robust risk stratification methods are lacking, we developed a risk score model to predict SCD in patients with ACHD: the PRospEctiVE study on implaNTable cardlOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD) risk score model.OBJECTIVE The purpose of this study was to prospectively study predicted SCD risk using the PREVENTION-ACHD risk score model and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF) rates in patients with ACHD.METHODS The PREVENTION-ACHD risk score model assigns 1 point each to coronary artery disease, New York Heart Association class II/III heart failure, supraventricular tachycardia, systemic ejection fraction < 40%, subpulmonary ejection fraction < 40%, QRS duration >= 120 ms, and QT dispersion >= 70 ms. SCD risk was calculated for each patient. An annual predicted risk of >= 3% constituted high risk. The primary outcome was SCD or VT/VF after 2 years. The secondary outcome was SCD.RESULTS The study included 783 consecutive patients with ACHD (n=239 (31%) left-sided lesions; n=138 (18%) tetralogy of Fallot; n=108 (14%) dosed atrial septal defect; median age 36 years; interquartile range 28-47 years; n=401 (51%) men). The PREVENTION-ACHD risk score modelidentified 58 high-risk patients. Eight patients (4 at high risk) experienced the primary outcome. The Kaplan-Meier estimates were 7% (95% confidence interval [CI] 0.1%-13.3%) in the high-risk group and 0.6% (95% CI 0.0%-1.1%) in the low-risk group (hazard ratio 12.5; 95% CI 3.1-50.9; P < .001). The risk score model's sensitivity was 0.5 and specificity 93, resulting in a C-statistic of 0.75 (95% CI 0.57-0.90). The hazard ratio for SCD was 12.4 (95% CI 1.8-88.1) (P = .01); the sensitivity and specificity were 0.5 and 0.92, and the C-statistic was 0.81 (95% CI 0.67-0.95).CONCLUSION The PREVENTION-ACHD risk score model provides greater accuracy in SCD or VT/VF risk stratification as compared with current guideline indications and identifies patients with ACHD who may benefit from preventive implantable cardioverterdefibrillator implantation.
KW - Adult congenital heart disease
KW - Implantable cardioverter-defibrillator
KW - Primary prevention
KW - Risk score
KW - Risk stratification
KW - Sudden cardiac death
KW - PREDICTION MODEL
KW - ARRHYTHMIAS
KW - MORTALITY
U2 - 10.1016/j.hrthm.2021.01.009
DO - 10.1016/j.hrthm.2021.01.009
M3 - Article
SN - 1547-5271
VL - 18
SP - 785
EP - 792
JO - Heart Rhythm
JF - Heart Rhythm
IS - 5
ER -