Background: Idiopathic pulmonary arterial hypertension (IPAH) is a progressive disorder that usually culminates in right ventricular failure and death without treatment.
Objective: To assess mortality trends by race and gender for idiopathic pulmonary arterial hypertension in the United States from 1994-1998.
Methods: The U.S. National Center for Health Statistics data for the years 1994-1998 was reviewed for deaths in which the underlying cause was primary pulmonary hypertension (ICD-9 code 416.0), now known as IPAH. The age, gender, race and state of residence of the deceased were abstracted from the Centers for Disease Control Wonder System (http://wonder.cdc.gov). Average annual age-adjusted region-, race- and gender-specific rates were then calculated.
Results: African-American women demonstrated the highest mortality rates for IPAH across all age groups compared to other racial and gender groups. No geographical differences in mortality rates were noted. An increase in mortality rates with advancing age was observed in all racial and gender groups, with the highest mortality rates for IPAH noted in the elderly.
Discussion: African Americans with IPAH exhibit a substantially increased mortality compared with Caucasians, particularly African-American women.