Hemodynamic Predictors of Survival in Scleroderma-related Pulmonary Arterial Hypertension. Scientific Publication

RATIONALE
Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a poorer prognosis compared to 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 SSc patients (64 women and 12 men; mean age 61 +/- 11 years) 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.

RESULTS

Forty (53%) patients were in WHO functional class III or IV. Mean pulmonary artery pressure was 41 +/- 11 mmHg, pulmonary vascular resistance (PVR) was 8.6 +/- 5.6 WU, and cardiac index (CI) 2.4 +/- 0.7 L/min/m(2). 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 (HR 1.10 [95%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/1.73m(2) portended a 3-fold 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.

CITATION Am J Respir Crit Care Med. 2010 Jul 15;182(2):252-60