Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension

Aránzazu Campo, Stephen C. Mathai, Jérôme Le Pavec, Ari L. Zaiman, Laura K. Hummers, Danielle Boyce, Traci Housten, Hunter C. Champion, Noah Lechtzin, Fredrick M. Wigley, Reda E. Girgis, Paul M. Hassoun

Research output: Contribution to journalArticlepeer-review

161 Scopus citations


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 mm Hg, pulmonary vascular resistance (PVR) was 8.6±5.6 Wood units, and cardiac index was 2.4±0.7 L/min/m2. Median follow-up time was 36 months, with 42 deaths observed. Survival estimates were 85%, 72%, 67%, 50%, and 36% at 1, 2, 3, 4, and 5 years, respectively. Multivariate analysis identified PVR (hazard ratio [HR], 1.10; 95% confidence interval [CI], 1.03-1.18; P < 0.01), stroke volume index (HR, 0.94; 95% CI, 0.89-0.99; P = 0.02), and pulmonary arterial capacitance (HR, 0.43;95%CI, 0.20-0.91; P= 0.03) as strong predictors of survival. An estimated glomerular filtration rate less than 60 ml/min/1.73 m2 portended a threefold risk of mortality. Conclusions: Our results suggest that specific components of right ventricular dysfunction and renal impairment contribute to increased mortality in SSc-PAH. Understanding the mechanisms of right ventricular dysfunction in response to increased afterload should lead to improved targeted therapy in these patients.

Original languageEnglish (US)
Pages (from-to)252-260
Number of pages9
JournalAmerican journal of respiratory and critical care medicine
Issue number2
StatePublished - Jul 15 2010

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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