Novel equation effective at predicting survival in pulmonary arterial hypertension
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A newly formed equation that can predict survival in patients with pulmonary arterial hypertension was more effective than the equation traditionally used by the National Institutes of Health to predict survival, study results suggested.
Researchers examined 576 patients in the Pulmonary Hypertension Connection registry diagnosed with PAH from 1991 to 2007. In patients with idiopathic, familial and anorexigen-associated PAH (n=247), the researchers compared observed survival with NIH equation-predicted survival. The researchers then developed their own survival prediction equation using exponential regression analysis.
Among the 576 patients included in the overall study cohort, 307 (53%) died during the median follow-up period of 3.9 years. Observed survival was 86% at one year, 69% at three years and 61% at five years in the overall cohort. Median follow-up time in the subgroup of patients with idiopathic, familial and anorexigen-associated PAH was 4.9 years. During that time, 149 of 282 (53%) patients died. Observed survival in the subgroup for which hemodynamic data were available was 92% at one year, 75% at three years, 66% at five years and was higher compared with patients whose PAH was associated with connective tissue disease, congenital heart disease, portal hypertension and HIV (P=.03). The observed survival rates were higher than those predicted with the NIH equation (65% at one year, 43% at three years and 32% at five years; P<.0001 for all comparisons). The researchers also reported that patients diagnosed after 2002 had better survival than those diagnosed prior to 2002.
The take-home message here is that all of the previously quoted survival estimates do not currently apply, and with all of our noninvasive and invasive testing, we are now able to get a better picture of the person when they are diagnosed, Mardi Gomberg-Maitland, MD, an associate professor of medicine at the University of Chicago and researcher for the study, told Cardiology Today. The aim is to better treat them and to hopefully avoid lung transplantation. by Eric Raible
Thenappan T. Eur Respir J. 2009; doi:10.1183/09031936.00072709.