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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 Editor
Geraldine Finlay, MD


Human immunodeficiency virus-related pulmonary arterial hypertension (HIV-PAH) exists when pulmonary arterial hypertension (PAH) develops in a patient who has human immunodeficiency virus (HIV) infection and an alternative cause cannot be identified. More than 200 cases have been reported since its initial description in 1987 [1-4].

In this topic review, the classification, prevalence, pathogenesis, clinical presentation, diagnostic evaluation, and treatment of HIV-PAH are discussed. Pulmonary hypertension 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)".)


The World Health Organization (WHO) classifies patients with pulmonary hypertension into five groups based upon etiology (table 1) [5]. 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 pulmonary hypertension (group 2, 3, 4, and 5 PH). When all five groups are discussed collectively, the term PH is used. (See "Classification and prognosis of pulmonary hypertension in adults", section on 'Classification'.)

Human immunodeficiency virus-related pulmonary arterial hypertension (HIV-PAH) is a type of PAH. It is a member of the "associated with PAH" subgroup that includes diseases that have an increased prevalence of coexisting PAH (table 1) [5]. Also included in the subgroup is PAH associated with drugs and toxins, connective tissue diseases, portal hypertension, congenital heart disease, schistosomiasis, chronic hemolytic anemia, persistent pulmonary hypertension of the newborn, pulmonary veno-occlusive disease, and pulmonary capillary hemangiomatosis. (See "Portopulmonary hypertension" and "Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis".)


Human immunodeficiency virus-related pulmonary arterial hypertension (HIV-PAH) is a rare complication of HIV infection, occurring in approximately 1 out of every 200 HIV-infected patients (0.5 percent) [6-10]. This is 6- to 12-times greater than the prevalence of PAH in individuals without HIV infection.

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Literature review current through: Sep 2017. | This topic last updated: Feb 09, 2016.
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