MiscellaneousNew predictors of outcome in idiopathic pulmonary arterial hypertension
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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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.