What is the prevalence of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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The overall prevalence of pulmonary arterial hypertension (PAH) is difficult to determine given the disease’s heterogeneity and likely underdiagnosis.

Worldwide, schistosomiasis is likely the most prevalent cause of PAH, [5] with studies suggesting that over 7% of patients with hepatosplenic schistosomiasis have pulmonary hypertension. [6, 7] However, data registries in countries most burdened by schistosomiasis-related PAH are limited. [5]

Data registries in areas without endemic schistosomiasis such as the United States and Europe report a PAH prevalence ranging from 6.6-26 cases per million adults. [8] The majority of these cases are idiopathic. While approximately 10% are classified as heritable, it is likely that this number will increase with time as genetic testing becomes more widespread.

Studies have also estimated the prevalence of specific subgroups of PAH. An observational study of 277 patients with HIV infection found that 0.46% of patients had pulmonary hypertension. [9] In comparison with prior studies, [10] no change in prevalence rate was seen with modern highly active antiretroviral treatment (HAART). In scleroderma patients, the incidence has been estimated to be 6-60% of all patients, with the variance based on the extent of disease. [11]

Women are more likely to have PAH, with registries reporting a 65-80% female predominance of the disease. [8] Interestingly, while prior studies suggested a mean age of diagnosis in the thirties, current registries suggest a mean age of diagnosis in the fifties. [8] Although PAH can affect all races, data from the US REVEAL registry suggest a white predominance (73% white vs 12% African American, 9% Latino, and 3% Asian). [12]

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