TY - JOUR
T1 - Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension
AU - Argula, Rahul G.
AU - Karwa, Abhijit
AU - Lauer, Abigail
AU - Gregg, David
AU - Silver, Richard M.
AU - Feghali-Bostwick, Carol
AU - Schanpp, Lynn M.
AU - Egbert, Kim
AU - Usher, Bruce W.
AU - Ramakrishnan, Viswanathan
AU - Hassoun, Paul M.
AU - Strange, Charlie
N1 - Publisher Copyright:
Copyright © 2017 by the American Thoracic Society.
PY - 2017/5
Y1 - 2017/5
N2 - 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.
AB - 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.
KW - Disease progression
KW - Idiopathic pulmonary arterial hypertension
KW - Pulmonary arterial hypertension
KW - Right ventricle
KW - Systemic sclerosis-associated pulmonary arterial hypertension
UR - http://www.scopus.com/inward/record.url?scp=85018975332&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85018975332&partnerID=8YFLogxK
U2 - 10.1513/AnnalsATS.201608-655OC
DO - 10.1513/AnnalsATS.201608-655OC
M3 - Article
C2 - 28282243
AN - SCOPUS:85018975332
SN - 2325-6621
VL - 14
SP - 682
EP - 689
JO - Annals of the American Thoracic Society
JF - Annals of the American Thoracic Society
IS - 5
ER -