Novel Therapies for Patients With Systemic Right Ventricle

Area of Science:

• Cardiology
• Adult Congenital Heart Disease
• Cardiovascular Physiology

Background:

• Systemic right ventricular (RV) dysfunction is a critical prognostic factor in congenital heart disease (CHD), especially in patients with transposition of the great arteries (TGA) post-atrial switch and congenitally corrected TGA (ccTGA).
• Chronic pressure loading in these conditions leads to RV structural, mechanical, and electrical maladaptations, often culminating in heart failure.
• Effective management strategies for systemic RV failure in adult CHD are crucial for improving patient prognosis.

Purpose of the Study:

• To review and discuss novel therapeutic approaches for managing systemic RV failure in adult CHD patients.
• To evaluate the efficacy of current and emerging pharmacologic, device-based, and advanced heart failure therapies.
• To highlight the need for personalized treatment strategies tailored to individual patient characteristics.

Main Methods:

• Review of existing literature on therapeutic interventions for systemic RV dysfunction in adult CHD.
• Analysis of outcomes associated with traditional heart failure medications.
• Evaluation of emerging pharmacologic agents (ARNI, SGLT2 inhibitors), cardiac resynchronization therapy (CRT), mechanical circulatory support (MCS), and heart transplantation (HTx).

Main Results:

• Traditional heart failure medications show variable efficacy in systemic RV failure.
• Newer agents like angiotensin receptor-neprilysin inhibitors (ARNIs) and sodium-glucose cotransporter 2 inhibitors (SGLT2i) demonstrate potential for improving RV remodeling and function.
• CRT can benefit selected patients, while MCS and HTx are effective options for advanced stages of heart failure, with favorable long-term outcomes reported.

Conclusions:

• Management of systemic RV failure in adult CHD necessitates a personalized approach considering unique patient physiology and anatomy.
• Novel pharmacologic and device-based therapies offer potential improvements over traditional treatments, which have shown limited efficacy.
• Further research is essential to refine existing strategies and identify new therapeutic targets for systemic RV failure in this population.