A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle

Magalie Ladouceur*, Alexander Van De Bruaene, Robert Kauling, Werner Budts, Jolien Roos-Hesselink, Sandra Villagrá Albert, Inmaculada Sanchez Perez, Berardo Sarubbi, Flavia Fusco, Pastora Gallego, Maria Jose Rodriguez-Puras, Judith Bouchardy, Coralie Blanche, Tobias Rutz, Katja Prokselj, Fabien Labombarda, Laurence Iserin, Tom Wong, Michael A. Gatzoulis

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

2 Citations (Scopus)

Abstract

AIMS: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). METHODS AND RESULTS: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. CONCLUSION: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

Original languageEnglish
Pages (from-to)2685-2694
Number of pages10
JournalEuropean Heart Journal
Volume43
Issue number28
DOIs
Publication statusPublished - 21 Jul 2022

Bibliographical note

Funding
This work was supported by the Fédération Française de Cardiologie and
the Assistance Publique des Hôpitaux de Paris.

Publisher Copyright:
© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

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