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 1 2017

Fingerprint

Systemic Scleroderma
Pulmonary Hypertension
Vasodilator Agents
Right Ventricular Dysfunction
Therapeutics
Right Ventricular Function
Tricuspid Valve Insufficiency
Lung
Familial Primary Pulmonary Hypertension
Interstitial Lung Diseases
Patient Rights
Cardiac Catheterization
Vascular Diseases
Pulmonary Artery
Lung Diseases
Regression Analysis
Phenotype
Mortality

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)
  • Pulmonary and Respiratory Medicine

Cite this

Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension. / Argula, Rahul G.; Karwa, Abhijit; Lauer, Abigail; Gregg, David; Silver, Richard M.; Feghali-Bostwick, Carol; Schanpp, Lynn M.; Egbert, Kim; Usher, Bruce W.; Ramakrishnan, Viswanathan; Hassoun, Paul M; Strange, Charlie.

In: Annals of the American Thoracic Society, Vol. 14, No. 5, 01.05.2017, p. 682-689.

Research output: Contribution to journalArticle

Argula, Rahul G. ; Karwa, Abhijit ; Lauer, Abigail ; Gregg, David ; Silver, Richard M. ; Feghali-Bostwick, Carol ; Schanpp, Lynn M. ; Egbert, Kim ; Usher, Bruce W. ; Ramakrishnan, Viswanathan ; Hassoun, Paul M ; Strange, Charlie. / Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension. In: Annals of the American Thoracic Society. 2017 ; Vol. 14, No. 5. pp. 682-689.
@article{99c72b5a583c429888c57a5d4fcb2ce3,
title = "Differences in right ventricular functional changes during treatment between systemic sclerosis-associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension",
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.",
keywords = "Disease progression, Idiopathic pulmonary arterial hypertension, Pulmonary arterial hypertension, Right ventricle, Systemic sclerosis-associated pulmonary arterial hypertension",
author = "Argula, {Rahul G.} and Abhijit Karwa and Abigail Lauer and David Gregg and Silver, {Richard M.} and Carol Feghali-Bostwick and Schanpp, {Lynn M.} and Kim Egbert and Usher, {Bruce W.} and Viswanathan Ramakrishnan and Hassoun, {Paul M} and Charlie Strange",
year = "2017",
month = "5",
day = "1",
doi = "10.1513/AnnalsATS.201608-655OC",
language = "English (US)",
volume = "14",
pages = "682--689",
journal = "Annals of the American Thoracic Society",
issn = "2325-6621",
publisher = "American Thoracic Society",
number = "5",

}

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

PY - 2017/5/1

Y1 - 2017/5/1

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

VL - 14

SP - 682

EP - 689

JO - Annals of the American Thoracic Society

JF - Annals of the American Thoracic Society

SN - 2325-6621

IS - 5

ER -