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Survival of Patients with Pulmonary Arterial Hypertension (PAH) Associated with Anorexigens vs. Survival Predicted From NIH Registry on Primary Pulmonary Hypertension (PPH) Observations

J. W. Schmidt

E. W. Glissmeyer

B. L. Wojciechowski

D. Adams

C. Gregory Elliott


R. L. Jensen

Matthew Hegewald


J. Carlquist

M. B. Scholand

T. Liou

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Conference: 2008 International PHA Conference and Scientific Sessions

Release Date: 06.20.2008

Presentation Type: Abstracts

Schmidt J.W., Glissmeyer E.W., Wojciechowski B.L., Adams D., Elliott C.G., Jensen R.L. Hegewald M.J., Carlquist J., Scholand M.B., Liou T., Mead M.

LDS Hospital, University of Utah, Taueret Laboratories, LLC, USA

BACKGROUNDPrevious data characterized survival in patients with PPH (Ann Int Med 1991; 115: 343-349). Anorexigens have been associated with an increased risk for PAH, but survival in patients with PAH associated with anorexigens is not well described.

METHODSWe conducted a cohort study of 43 patients with PAH associated with anorexigens enrolled consecutively in the Utah Pulmonary Hypertension Genetics Project. The index date for calculating survival proportions was the date of diagnostic right heart catheterization and survival status was confirmed Oct 15, 2007. Kaplan-Meier estimates were compared to the probability of survival predicted by the equation derived from the NIH registry on PPH (Ann Int Med 1991; 115: 343-349).

RESULTS:  Kaplan-Meier estimated survival proportions are displayed in Figure 1. The cohort's predicted probability of survival was 0.68 in year 1, 0.57 in year 2, and 0.48 in year 3.

Survival of Patients with Pulmonary Arterial Hypertension (PAH) Associated with Anorexigens vs. Surv

CONCLUSIONS: The observed survival of this cohort of patients with PAH associated with anorexigens appears very different from their survival predicted by an equation derived from the NIH Registry. This difference may reflect secular trends in management, the use of PAH specific therapies, and/or differences in the natural history of PAH associated with anorexigens.