Scientific publications

Hemodynamic Predictors of Survival in Scleroderma-related Pulmonary Arterial Hypertension

Campo A [SP], Mathai SC, Le Pavec J, Zaiman AL, Hummers LK, Boyce D, Housten T, Champion HC, Lechtzin N, Wigley FM, Girgis RE, Hassoun PM.
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.

Magazine: American Journal of Respiratory and Critical Care Medicine

Date: Mar 25, 2010


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

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

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.


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.

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

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