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


METHODS
Retrospective cohort study.


METHODS
Tertiary care medical center.


METHODS
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.


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.
Kawut
Steven M
SM
Pulmonary, Allergy, and Critical Care, University of Pennsylvania School of Medicine, Philadelphia, USA. SK2097@columbia.edu
Taichman
Darren B
DB
Archer-Chicko
Christine L
CL
Palevsky
Harold I
HI
Kimmel
Stephen E
SE
D000328:Adult; D000368:Aged; D006328:Cardiac Catheterization; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery / Q000187:drug effects; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012306:Risk; D012595:Scleroderma, Systemic / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy; D015996:Survival Rate; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000008:administration & dosage
D014665:Vasodilator Agents