Novel Therapies for Patients With Systemic Right Ventricle

Systemic RV dysfunction is a significant prognostic factor in congenital heart disease, particularly affecting patients with transposition of the great arteries post-atrial switch and congenitally corrected transposition of the great arteries. These patients often experience chronic pressure loading conditions, leading to structural, mechanical, and electrical adaptations that can result in heart failure. This article reviews novel therapeutic approaches for managing systemic right ventricular failure in patients with adult congenital heart disease, focusing on pharmacologic, device-based, and advanced heart failure therapies. Traditional heart failure medications such as angiotensin converting enzyme inhibitors, angiotensin II receptor blockers, β-blockers, and mineralocorticoid receptor antagonists have shown mixed results in systemic right ventricular failure. However, newer agents like angiotensin receptor-neprilysin inhibitors and sodium-glucose cotransporter 2 inhibitors have demonstrated promising outcomes in improving cardiac remodeling and function. Cardiac resynchronization therapy has shown benefits in selected patients, particularly those with prior ventricular pacing. Mechanical circulatory support and heart transplantation remain viable options for advanced heart failure, with recent studies indicating favourable long-term outcomes. The management of systemic right ventricular failure requires a personalized approach, considering the unique anatomic and physiological characteristics of each patient. Although traditional therapies have limited efficacy, novel pharmacologic and device-based treatments might offer new hope for this patient population. Further research is needed to refine these strategies and identify new therapeutic targets.