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).


Journal

Heart rhythm
ISSN: 1556-3871
Titre abrégé: Heart Rhythm
Pays: United States
ID NLM: 101200317

Informations de publication

Date de publication:
05 2021
Historique:
received: 16 04 2020
revised: 27 12 2020
accepted: 10 01 2021
pubmed: 20 1 2021
medline: 19 1 2022
entrez: 19 1 2021
Statut: ppublish

Résumé

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. 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. 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. 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). 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.

Sections du résumé

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.

Identifiants

pubmed: 33465514
pii: S1547-5271(21)00029-1
doi: 10.1016/j.hrthm.2021.01.009
pii:
doi:

Types de publication

Journal Article Observational Study Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

785-792

Informations de copyright

Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Auteurs

Jim T Vehmeijer (JT)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Zeliha Koyak (Z)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Jan M Leerink (JM)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Aeilko H Zwinderman (AH)

Department of Clinical Epidemiology and Biostatistics, Amsterdam University Medical Centers/University of Amsterdam, The Netherlands.

Louise Harris (L)

Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center of Adults/University of Toronto, Toronto, Ontario, Canada.

Rafael Peinado (R)

Department of Cardiology, La Paz University Hospital/Autonomous University of Madrid, Madrid, Spain.

Erwin N Oechslin (EN)

Division of Cardiology, Peter Munk Cardiac Center, Toronto Congenital Cardiac Center of Adults/University of Toronto, Toronto, Ontario, Canada.

Daniëlle Robbers-Visser (D)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Maarten Groenink (M)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

S Matthijs Boekholdt (SM)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Robbert J de Winter (RJ)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

José M Oliver (JM)

Department of Cardiology, Gregorio Marañon University Hospital, CIBERCV, Madrid, Spain.

Berto J Bouma (BJ)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands.

Werner Budts (W)

Department of Cardiology, Universitair Ziekenhuis Leuven/Department of Cardiovascular Sciences, Katholieke Universiteit Leuven, Leuven, Belgium.

Isabelle C Van Gelder (IC)

Department of Cardiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Barbara J M Mulder (BJM)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands; Netherlands Heart Institute, Utrecht, The Netherlands.

Joris R de Groot (JR)

Department of Cardiology, Heart Center, Amsterdam University Medical Centers/University of Amsterdam, Amsterdam, The Netherlands. Electronic address: j.r.degroot@amsterdamumc.nl.

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