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)

Jim T. Vehmeijer, Zeliha Koyak, Jan M. Leerink, Aeilko H. Zwinderman, Louise Harris, Rafael Peinado, Erwin N. Oechslin, Daniëlle Robbers-Visser, Maarten Groenink, S. Matthijs Boekholdt, Robbert J. de Winter, José M. Oliver, Berto J. Bouma, Werner Budts, Isabelle C. van Gelder, Barbara J. M. Mulder, Joris R. de Groot

Research output: Contribution to journalArticleAcademicpeer-review

19 Citations (Scopus)

Abstract

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 cardIOverter 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%) closed atrial septal defect; median age 36 years; interquartile range 28–47 years; n=401 (51%) men). The PREVENTION-ACHD risk score model identified 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 0.93, 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 cardioverter-defibrillator implantation.

Original languageEnglish
Pages (from-to)785-792
Number of pages8
JournalHeart Rhythm
Volume18
Issue number5
DOIs
Publication statusPublished - 1 May 2021

Keywords

  • Adult congenital heart disease
  • Implantable cardioverter-defibrillator
  • Primary prevention
  • Risk score
  • Risk stratification
  • Sudden cardiac death

Cite this