The systemic right ventricle in adult congenital heart disease: why is it still such a challenge and is there any hope on the horizon?

PURPOSE OF REVIEW: Adult congenital heart disease patients with systemic right ventricle comprise a distinctly clinically challenging group of patients with increased morbidity and mortality. This article aims to review the different subgroups, most common complications and different treatment strategies. RECENT FINDINGS: Most commons long-term complications include heart failure and arrhythmias. Heart failure medical therapy treatments include several new agents, which show promise in systemic right ventricle patients. In addition, interventional therapies to mitigate atrioventricular valve regurgitation, baffle/conduit stenosis are discussed. Furthermore, several electrophysiological approaches to manage tachyarrhythmias as well as bradycardias are discussed. There is ongoing excitement on the new medical as well as interventional therapies that could provide benefit in additional to standard goal-directed medical therapy. SUMMARY: There is an array of medications as well as interventions aimed to treat patients with systemic right ventricle with limited benefits. A multidisciplinary approach with a prudent combination of such therapies to maximize benefit is imperative. This article reviews the data supporting such therapies.