The role of the right ventricle in pulmonary arterial hypertension

Abstract

Although changes in the pulmonary vasculature are the primary cause of pulmonary arterial hypertension (PAH), severity of symptoms and survival are strongly associated with right ventricular function, and right heart failure is the main cause of death in patients with PAH. Echocardiography and cardiac magnetic resonance imaging allow noninvasive evaluation of right ventricular function and structure, and a number of indices have been shown to have potential prognostic value in PAH. Given the importance of the right ventricle in PAH, preservation and improvement of its function should be important aspects of therapy; however, there are currently few data specifically related to this aspect of treatment response. Simple, reproducible, noninvasive measures of right ventricular function would help to improve the management of patients with PAH, and to provide tools with which to help establish the optimal therapeutic approach to manage not only the effects of the disease on the pulmonary vasculature, but also to support and improve right ventricular function.

Figure 1.

Kaplan–Meier survival curves for echocardiographic predictors of outcomes. Reproduced from [25] with permission from the publisher.

Figure 2.

Kaplan–Meier survival curves for baseline cardiac magnetic resonance imaging variables according to the median value in patients with pulmonary hypertension. SVI: stroke volume index; RV: right ventricular; RVEDVI: RV end-diastolic volume index; LVEDVI: left ventricular end-diastolic volume index. Reproduced from [48] with permission from the publisher.

Figure 3.

Meta-analysis of pulmonary arterial hypertension trials by pump function. ΔSVI: change in indexed stroke volume; ΔP̄_pa: change in mean pulmonary arterial pressure; PVR: pulmonary vascular resistance. Reproduced from [12] with permission.