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Ann Am Thorac Soc. 2017 May;14(5):682-689. doi: 10.1513/AnnalsATS.201608-655OC.

Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension.

Author information

1
1 Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine.
2
2 Division of Internal Medicine.
3
3 Department of Public Health Sciences.
4
4 Division of Cardiology, and.
5
5 Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; and.
6
6 Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.

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 (-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.

KEYWORDS:

disease progression; idiopathic pulmonary arterial hypertension; pulmonary arterial hypertension; right ventricle; systemic sclerosis–associated pulmonary arterial hypertension

PMID:
28282243
PMCID:
PMC5802595
DOI:
10.1513/AnnalsATS.201608-655OC
[Indexed for MEDLINE]
Free PMC Article

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