Abstract
Rationale
Right ventricular (RV) failure is the main mortality determinant in idiopathic pulmonary arterial hypertension (iPAH). However, little is known about adaptive and maladaptive changes in RV performance during disease progression. This study aims to characterize global RV function in mild, intermediate and severe iPAH based on patient survival duration from iPAH diagnosis.
Methods
Right heart catheterization and MRI were performed in 67 individuals (Con=10, Mild=28, Intermediate=11, Severe=18). iPAH severity was defined as: less than 6 months survival (severe), between 1-3 years survival (intermediate) and more than 5 years survival (mild). RV systolic elastance (Ees), arterial elastance (Ea), RV coupling (Ees/Ea) and diastolic elastance (Ed) were quantified by pressure-volume analysis.
Results
RV systolic and diastolic elastance were significantly increased in iPAH compared to controls. RV coupling was maintained in mild and intermediate iPAH, however significantly decreased in severe iPAH. RV diastolic elastance was preserved during mild iPAH, but significantly increased in intermediate and severe disease.
Conclusions
Systolic RV adaptation to the increased afterload is only compromised in severe iPAH. However, RV diastolic impairment occurs already from intermediate iPAH.
- © 2013 ERS