Role of subpulmonary right ventricle in sudden cardiac death in adults with congenital heart disease

Satoshi Kawada; Praloy Chakraborty; Jayant Kakarla; Jared Nanthakumar; Blandine Mondésert; Paul Khairy; Krishnakumar Nair

doi:10.1016/j.hrthm.2024.08.016

Role of subpulmonary right ventricle in sudden cardiac death in adults with congenital heart disease

Heart Rhythm. 2024 Aug 8:S1547-5271(24)03127-8. doi: 10.1016/j.hrthm.2024.08.016. Online ahead of print.

Authors

Satoshi Kawada¹, Praloy Chakraborty¹, Jayant Kakarla¹, Jared Nanthakumar², Blandine Mondésert³, Paul Khairy³, Krishnakumar Nair⁴

Affiliations

¹ Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, and University of Toronto, Toronto, Ontario, Canada.
² Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, and University of Toronto, Toronto, Ontario, Canada; Michael De Groote School of Medicine, McMaster University, Hamilton, Ontario, Canada.
³ Adult Congenital Heart Disease Centre, Department of Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada.
⁴ Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Health Network Toronto, and University of Toronto, Toronto, Ontario, Canada. Electronic address: [email protected].

PMID: 39127230
DOI: 10.1016/j.hrthm.2024.08.016

Abstract

Despite improved childhood survival of congenital heart disease (CHD) as a result of advances in management, late-onset sudden cardiac death (SCD) from malignant ventricular arrhythmias remains a leading cause of mortality in adults with CHD. Preventing SCD in these patients requires an understanding of the underlying pathophysiological mechanisms. Many CHD patients experience significant hemodynamic stress on the subpulmonary right ventricle (RV), leading to pathologic remodeling. Unlike acquired heart disease in which left ventricular pathology is prevalent, RV pathologies are crucial in the SCD pathogenesis in CHD patients. This review examines the mechanisms and management of SCD related to subpulmonary RV pathologies in CHD patients.

Keywords: Congenital heart disease; Electrophysiological remodeling; Subpulmonary right ventricle; Sudden cardiac death; Ventricular arrhythmia.

Publication types

Review