Managing and predicting electrical and mechanical failure in the systemic right ventricle

Adults with congenital heart disease increasingly survive into later adulthood, exposing the long-term limitations of a morphologic right ventricle supporting the systemic circulation. Heart failure and arrhythmias are common, frequently coexist, and represent leading causes of morbidity and mortality in this population. This review examines the mechanisms linking systemic right ventricular dysfunction and arrhythmogenesis and their implications for contemporary management.

Recent literature highlights that chronic systemic loading of the right ventricle promotes maladaptive structural, electrical, and inflammatory remodeling, creating a substrate for atrial and ventricular arrhythmias. Conversely, arrhythmias exacerbate systemic right ventricular dysfunction through loss of atrioventricular synchrony, tachycardia-mediated impairment, dyssynchrony, and hypoxemia, mechanisms to which systemic right ventricular physiologies are particularly susceptible. Advances in catheter ablation, device therapy, cardiac resynchronization, and conduction system pacing have expanded therapeutic options, although evidence guiding patient selection and timing remains limited.

Failure of the systemic right ventricle and arrhythmias are tightly interwoven processes that evolve over time. Integrating mechanistic insights with longitudinal imaging, electrophysiologic phenotyping, and individualized risk assessment may improve arrhythmia management, refine device strategies, and support more personalized approaches to preserving systemic right ventricular function.