Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension

Rahul G. Argula, Abhijit Karwa, Abigail Lauer, David Gregg, Richard M. Silver, Carol Feghali-Bostwick, Lynn M. Schanpp, Kim Egbert, Bruce W. Usher, Viswanathan Ramakrishnan, Paul M. Hassoun, Charlie Strange

Research output: Contribution to journalArticle

Abstract

Rationale: Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies. Objectives: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction. Methods: Retrospective analysis of echocardiographic data in 13 patients with SSc-PAH and 11 patients with IPAH was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy. All patients had right heart catheterization-confirmed pulmonary arterial hypertension as well as complete baseline (at the time of diagnosis) and follow-up (most recent) echocardiograms. We excluded patients with significant scleroderma-associated interstitial lung disease. Adjusting for time of follow-up and disease duration, we performed mixed model regression analyses comparing the changes between the two groups for different echocardiographic variables: tricuspid annular plane systolic excursion, tricuspid regurgitation jet velocity, right atrial area, and RV diameter. Results: The mean ages for the SSc-PAH and IPAH groups were 60.8 and 48.2 years, respectively. The mean follow-up periods for the two groups were 3.8 and 1.95 years, respectively. Tricuspid annular plane systolic excursion did not improve in patients with SSc-PAH, whereas it increased in the patients with IPAH (20.38 mm, P = 0.87; vs. 15.6 mm, P = 0.02). The other echocardiographic variables showed a trend toward worsening in the SSc-PAH group and improvement in the IPAH group. Conclusions: Our results indicate that, in patients with SSc-PAH, echocardiographic RV function does not improve over time compared with that of patients with IPAH, despite institution of pulmonary artery vasodilator therapies.

Original languageEnglish (US)
Pages (from-to)682-689
Number of pages8
JournalAnnals of the American Thoracic Society
Volume14
Issue number5
DOIs
StatePublished - May 2017

Keywords

  • Disease progression
  • Idiopathic pulmonary arterial hypertension
  • Pulmonary arterial hypertension
  • Right ventricle
  • Systemic sclerosis-associated pulmonary arterial hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Fingerprint Dive into the research topics of 'Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension'. Together they form a unique fingerprint.

  • Cite this