Department of Pulmonary Diseases, Institute for Cardiovascular Research ICaR-VU, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
AIMS: This study investigated the relationship between right ventricular (RV) structure and function and survival in idiopathic pulmonary arterial hypertension (IPAH). METHODS AND RESULTS: In 64 patients, cardiac magnetic resonance, right heart catheterization, and the six-minute walk test (6MWT) were performed at baseline and after 1-year follow-up. RV structure and function were analysed as predictors of mortality. During a mean follow-up of 32 months, 19 patients died. A low stroke volume (SV), RV dilatation, and impaired left ventricular (LV) filling independently predicted mortality. In addition, a further decrease in SV, progressive RV dilatation, and further decrease in LV end-diastolic volume (LVEDV) at 1-year follow-up were the strongest predictors of mortality. According to Kaplan-Meier survival curves, survival was lower in patients with an inframedian SV index <or= 25 mL/m(2), a supramedian RV end-diastolic volume index >or= 84 mL/m(2), and an inframedian LVEDV<or=40 mL/m(2). CONCLUSIONS: The RV contains prognostic information in IPAH. A large RV volume, low SV, and a reduced LV volume are strong independent predictors of mortality and treatment failure.