RT Journal Article
SR Electronic
T1 Load-independent right ventricular function and idiopathic pulmonary arterial hypertension severity
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P3404
VO 42
IS Suppl 57
A1 Pia Trip
A1 Silvia Rain
A1 Harm-Jan Bogaard
A1 Nico Westerhof
A1 Jolanda van der Velden
A1 Frances S. de Man
A1 Anton Vonk-Noordegraaf
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/P3404.abstract
AB RationaleRight 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.MethodsRight 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.ResultsRV 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.ConclusionsSystolic RV adaptation to the increased afterload is only compromised in severe iPAH. However, RV diastolic impairment occurs already from intermediate iPAH.