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Pulmonary arterial hypertension associated with human immunodeficiency virus

Michael Ieong, MD
Harrison W Farber, MD
Section Editor
Jess Mandel, MD
Deputy Editors
Geraldine Finlay, MD
Jennifer Mitty, MD, MPH


Human immunodeficiency virus (HIV) infection can be complicated by pulmonary hypertension (PH). The World Health Organization (WHO) classifies patients with PH into five groups based upon etiology (table 1) [1]. Patients in the first group are considered to have pulmonary arterial hypertension (group 1 PAH), while patients in the remaining four groups are considered to have PH (group 2, 3, 4, and 5 PH). When all five groups are discussed collectively, the term PH is used. HIV-related PAH (HIV-PAH) belongs to group 1.

In this topic review, the prevalence, pathogenesis, clinical diagnostic evaluation, and treatment of HIV-PAH are discussed. PH that is unrelated to HIV infection is discussed separately. (See "Classification and prognosis of pulmonary hypertension in adults" and "Treatment of pulmonary hypertension in adults" and "Clinical features and diagnosis of pulmonary hypertension in adults" and "The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1)".)


HIV-PAH is a rare complication of HIV infection, occurring in approximately 1 out of every 200 HIV-infected patients (0.5 percent) [2-11]. This is 100 to 1000-times greater than the prevalence of PAH in individuals without HIV infection. However, studies likely underestimate the true prevalence of HIV-PAH because patients with asymptomatic PAH are not included. (See "The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1)", section on 'Epidemiology'.)

Best supporting this prevalence is a prospective study of 7648 patients with HIV, which found a prevalence of right-heart catheterization-confirmed HIV-PAH of 0.5 percent [6]. This study, which was performed from 2004 to 2005 (after the introduction of potent antiretroviral therapy [ART]), reported a similar prevalence to that which was reported in studies performed prior to the use of effective ART, suggesting that ART has not altered the prevalence of HIV-PAH [6,10]. (See 'Antiretroviral therapy' below.)

Smaller cohort studies have demonstrated prevalence rates of HIV-PAH as high as 2.6 to 14 percent using echocardiography [9,12-14]. However, the observational design of many of these studies and the limitations of echocardiography in the diagnosis of PAH make such assessments of prevalence unreliable [15].

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Literature review current through: Dec 2017. | This topic last updated: Nov 21, 2017.
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