Abstract Patients with a systemic right ventricle (sRV)—including those with transposition of the great arteries (TGA) following atrial switch repair (Mustard or Senning procedures) and congenitally corrected TGA—represent one of the greatest challenges in the management of adults with congenital heart disease (ACHD). The right ventricle is not anatomically designed to sustain systemic pressure over a lifetime. As a result, sRV failure is almost inevitable and follows a progressive, multifactorial process that initiates with maladaptive functional and structural changes. In later stages, arrhythmias, conduction delays, and pacing-induced dyssynchrony emerge, accelerating deterioration. Finally, lifestyle and environmental factors such as inactivity, obesity, or pregnancy may trigger clinical decompensation. Recognizing where a patient lies along this continuum and identifying those at highest risk is essential to anticipate deterioration and implement timely interventions. Despite significant advances in the management of ACHD, important gaps in knowledge and research remain regarding the sRV, particularly in understanding its long-term adaptation, predictors of failure, optimal medical therapy, prevention of dyssynchrony, and timing for transplant. We herewith summarize latest evidences on prognostic determinants, multimodality imaging, heart failure therapy, electrical therapy for heart failure, mechanical circulatory systems, and heart transplant in sRV.
Fusco et al. (Tue,) studied this question.