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Published ahead of print on February 25, 2003, doi:10.1164/rccm.200204-330OC

Am. J. Respir. Crit. Care Med., Volume 167, Number 10, May 2003, 1433-1439

A more recent version of this article appeared on May 15, 2003
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Submitted on April 23, 2002
Accepted on February 11, 2003

Prognostic factors for survival in HIV-associated pulmonary arterial hypertension

Hilario Nunes1, Marc Humbert1*, Olivier Sitbon1, Jane H Morse2, Zemin Deng3, James A Knowles3, Catherine Le Gall1, Florence Parent1, Gilles Garcia1, Philippe Herve1, Robyn J Barst2, and Gerald Simonneau1

1 Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Reanimation Respiratoire, Hopital Antoine Beclere, Assistance Publique -Hopitaux de Paris, Universite Paris-Sud, Clamart, France, 2 Medicine, Pediatrics and Psychiatry, Columbia University College of Physicians and Surgeons, New York, NY, USA, 3 Medicine, Pediatrics and Psychiatry, Columbia University College of Physicians and Surgeons, New York, NY, USA; The New York State Psychiatric Institute, New York, NY, USA

* To whom correspondence should be addressed. E-mail: humbert{at}ipsc.u-psud.fr.

We report a large monocentric case series of 82 patients with HIV-associated pulmonary arterial hypertension. No germline mutations of the PPH1 gene (bone morphogenetic protein receptor-II) were found in any of the 19 patients tested. Pulmonary arterial hypertension was the direct cause of death in 72% of cases. Survival rates of the overall population at 1, 2 and 3 years were 73%, 60% and 47%, respectively. Survival was significantly poorer in patients in New York Heart Association functional class III-IV at the time of diagnosis, as compared to those in functional class I-II with respective rates of 60%, 45% and 28% versus 100%, 90%, 84% at 1, 2 and 3 years (p<0.0001). Subsequently, we analyzed prognostic factors in patients in functional class III-IV. Univariate analysis indicated that CD4 lymphocyte count > 212 cells.mm-3, the use of combination antiretroviral therapy and epoprostenol infusion were related with a better survival. On multivariate analysis only CD4 lymphocyte count was an independent predictor of survival, presumably because combination antiretroviral therapy and epoprostenol infusion were strongly linked in our patient population. These results suggest that patients with severe HIV-associated pulmonary arterial hypertension should be considered for long-term epoprostenol infusion in association with combination antiretroviral therapy.
Key words: Epoprostenol, Antiretroviral therapy, Human immunodeficiency virus, Pulmonary hypertension




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