Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension.

ABSTRACT:

Rationale

Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a poorer prognosis compared with other forms of PAH for reasons that remain unexplained.

Objectives

To identify risk factors of mortality in a well-characterized cohort of patients with PAH related to systemic sclerosis (SSc-PAH).

Methods

Seventy-six consecutive patients with SSc (64 women and 12 men; mean age 61 +/- 11 yr) were diagnosed with PAH by heart catheterization in a single center, starting in January 2000, and followed over time. Kaplan-Meier estimates were calculated and mortality risk factors were analyzed.

Measurements and main results

Forty (53%) patients were in World Health Organization functional class III or IV. Mean pulmonary artery pressure was 41 +/- 11

SUBMITTER: Campo A 

PROVIDER: S-EPMC2913238 | biostudies-literature | 2010 Jul

REPOSITORIES: biostudies-literature