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Published ahead of print on November 21, 2002, doi:10.1164/rccm.200204-333OC

Am. J. Respir. Crit. Care Med., Volume 167, Number 4, February 2003, 580-586

A more recent version of this article appeared on February 15, 2003
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Submitted on April 18, 2002
Accepted on November 15, 2002

Outcome in 91 Consecutive Patients with Pulmonary Arterial Hypertension Receiving Epoprostenol

Karl P Kuhn1, Daniel W Byrne2, Patrick W Arbogast3, Thomas P Doyle4, James E Loyd1, and Ivan M Robbins1*

1 Center for Lung Research, Division of Allergy, Pulmonary and Critical Care MedicineMedicine, Vanderbilt University, Nashville, TN, USA, 2 Division of General Internal Medicine, General Clinical Research Center, Department of Medicine, Vanderbilt University, Nashville, TN, USA, 3 Division of Biostatistics, Department of Preventive Medicine, Vanderbilt University, Nashville, TN, USA, 4 Division of Pediatric Cardiology, Vanderbilt University, Nashville, TN, USA

* To whom correspondence should be addressed. E-mail: ivan.robbins{at}vanderbilt.edu.

Epoprostenol has markedly improved the treatment of pulmonary arterial hypertension (PAH), although predictors of outcome with epoprostenol are not well characterized. From June 1995 through August 2001, 91 patients with PAH were treated with epoprostenol at our institution. We analyzed the effects of long-term epoprostenol treatment to determine features associated with outcome. Predictors of worse outcome included older age of disease onset (Hazard Ratio 3.2, 95% Confidence Interval 1.32 to 7.76 for patients above the median age of 44 years), World Health Organization functional class IV, either at baseline or follow-up, (3.07, 1.42 to 6.62 compared to functional class I, II, and III), and Scleroderma Spectrum of Disease (SSD) (2.32, 1.08 to 4.99). There were no baseline or follow-up hemodynamic factors predictive of outcome. Our results indicate that treatment with epoprostenol improves survival in patients with Primary Pulmonary Hypertension (PPH) compared to that predicted by the NIH PPH Registry's survival equation, and that their survival is significantly better than that of patients with SSD (p=0.001). Older patients treated with epoprostenol have significantly shorter survival, regardless of etiology.
Key words: pulmonary hypertension, mortality, flolan




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