Miscellaneous
New predictors of outcome in idiopathic pulmonary arterial hypertension

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Idiopathic pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. New therapies have improved the outcome of this condition; accordingly, the factors that determine outcome may have changed. We aimed to identify determinants of survival in a cohort of consecutive patients with PAH: which was idiopathic, familial, or associated with anorexigen use. We performed a retrospective cohort study of 84 consecutive patients with PAH who underwent initial evaluation at our center from January 1994 to June 2002. The primary outcome was death or lung transplantation. Survival at 1, 2, and 3 years was 87%, 75%, and 61%, respectively. Multivariate analysis showed that being of African-American or Asian descent was associated with an increased risk of death. Warfarin use was associated with a reduced risk of death. Higher serum albumin and cardiac index and acute vasoreactivity were independently associated with improved survival. These data suggest that the determinants of outcome have changed. Race is identified as a new risk factor, which may be attributable to biologic or socioeconomic differences. Cardiac function and acute reactivity of the pulmonary vascular bed remain strong independent predictors of outcome.

Section snippets

Study design

We performed a retrospective cohort study of all consecutive adult patients with idiopathic PAH who underwent initial evaluation from January 1994 to June 2002 with follow-up through June 2003 at our center. The study was approved by the institutional review board.

Study subjects

To assemble the cohort, we queried the Data Warehouse at our institution for all patients with an International Classification of Diseases-Ninth Revision code for primary or secondary pulmonary hypertension who were assessed by

Results

Our cohort was composed of 84 newly diagnosed patients with PAH (mean age 42 ± 14 years). Sixty-eight (81%) were women. The cohort included 69 whites (82%, of whom 9 were Hispanic and 60 were non-Hispanic) and 15 non-whites (18%, 9 Asians and 6 African-Americans). Sixty-six patients (78%) had idiopathic PAH, 14 (17%) had familial PAH, and 4 (5%) had PAH related to anorexigen use. The median time between the performance of echocardiography and/or cardiac catheterization consistent with a

Discussion

This is the largest study of outcomes in patients with idiopathic PAH treated with currently approved PAH therapies. We have shown that cardiac index, acute vasoreactivity, race, and serum albumin independently predict transplant-free survival in patients with idiopathic PAH. Although warfarin use was associated with increased survival, the use of other PAH medications was not. These results differ from those of recently published studies.12, 13, 14, 15 Our data also demonstrate that right

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  • Cited by (0)

    Dr. Kawut was supported by Grant HL67771 from the National Institutes of Health, Bethesda, Maryland, and the Florence and Herbert Irving Clinical Research Career Award, New York, New York.

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