title: Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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 (-0.38 mm, P = 0.87; vs. +5.6 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.
authors.affiliation: 1 Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine.
authors.forename: Rahul G
authors.initials: RG
authors.lastname: Argula
authors.affiliation: 2 Division of Internal Medicine.
authors.forename: Abhijit
authors.initials: A
authors.lastname: Karwa
authors.affiliation: 3 Department of Public Health Sciences.
authors.forename: Abigail
authors.initials: A
authors.lastname: Lauer
authors.affiliation: 4 Division of Cardiology, and.
authors.forename: David
authors.initials: D
authors.lastname: Gregg
authors.affiliation: 5 Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; and.
authors.forename: Richard M
authors.initials: RM
authors.lastname: Silver
authors.affiliation: 5 Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; and.
authors.forename: Carol
authors.initials: C
authors.lastname: Feghali-Bostwick
authors.affiliation: 1 Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine.
authors.forename: Lynn M
authors.initials: LM
authors.lastname: Schanpp
authors.affiliation: 1 Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine.
authors.forename: Kim
authors.initials: K
authors.lastname: Egbert
authors.affiliation: 4 Division of Cardiology, and.
authors.forename: Bruce W
authors.initials: BW
authors.lastname: Usher
authors.affiliation: 3 Department of Public Health Sciences.
authors.forename: Viswanathan
authors.initials: V
authors.lastname: Ramakrishnan
authors.affiliation: 6 Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Hassoun
authors.affiliation: 1 Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine.
authors.forename: Charlie
authors.initials: C
authors.lastname: Strange
mesh_terms: D000328:Adult; D000368:Aged; D006328:Cardiac Catheterization; D016208:Databases, Factual; D018450:Disease Progression; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension / Q000503:physiopathology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*; D013022:South Carolina; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016278:Ventricular Function, Right
keywords: disease progression; idiopathic pulmonary arterial hypertension; pulmonary arterial hypertension; right ventricle; systemic sclerosis–associated pulmonary arterial hypertension