Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis.

Abstract:

STUDY OBJECTIVES:The goal of this study was to determine whether the survival of patients with pulmonary hypertension related to systemic sclerosis (SScPH) was different from that of patients with other forms of pulmonary arterial hypertension. DESIGN:Retrospective cohort study. SETTING:Tertiary care medical center. PATIENTS:Our cohort was composed of 33 patients with pulmonary hypertension that is sporadic, familial, or related to anorexigen use (PPH) and 22 patients with SScPH who underwent initial pulmonary artery catheterization and vasodilator study at our center between January 1997 and June 2001. MEASUREMENTS AND RESULTS:Patients with SScPH had somewhat lower percentage of predicted lung volumes than patients with PPH (total lung capacity, 80% vs 92%; p = 0.06) and had lower percentage of predicted diffusion capacity of the lung for carbon monoxide (42% vs 68%; p = 0.0002). Right atrial pressure, pulmonary artery pressure, and cardiac index were similar between the groups. Patients with SScPH and PPH were treated with usual medical therapies, such as digoxin, warfarin, and continuous IV epoprostenol. Despite these similarities, the risk of death in patients with SScPH was higher than in patients with PPH (unadjusted hazard ratio, 2.9; 95% confidence interval, 1.1 to 7.8; p = 0.03). This increased risk appeared to persist after adjustment for a variety of demographic, hemodynamic, or treatment variables. CONCLUSIONS:Despite having similar hemodynamics, patients with SScPH have a higher risk of death than patients with PPH. Future studies of the mechanism and therapy of pulmonary arterial hypertension should focus on the distinctions between the different forms of this disease.

journal_name

Chest

journal_title

Chest

authors

Kawut SM,Taichman DB,Archer-Chicko CL,Palevsky HI,Kimmel SE

doi

10.1378/chest.123.2.344

subject

Has Abstract

pub_date

2003-02-01 00:00:00

pages

344-50

issue

2

eissn

0012-3692

issn

1931-3543

pii

S0012-3692(15)32440-5

journal_volume

123

pub_type

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