HIV-associated pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a severe complication of human immunodeficiency virus (HIV) infection. PAH associated with HIV infection (PAH-HIV) has gained importance following the improved survival of HIV-infected patients with the use of highly active antiretroviral therapy (HAART). PAH-HIV is associated with a particularly poor prognosis and decreased survival relative to HIV-infected patients without PAH, and patients with PAH-HIV tend to die from the effects of PAH rather than as a result of their HIV infection. The clinical and pathological findings in PAH-HIV share many features with the idiopathic form of the disease (iPAH). Since specific guidelines for optimal management of PAH-HIV are still lacking, diagnostic and therapeutic algorithms largely follow recommendations for iPAH. Treatment with epoprostenol has been reported to provide benefit in some cases but is associated with a range of problems linked to the need for continuous intravenous infusion. The dual endothelin receptor antagonist bosentan has proved to be effective in PAH-HIV without affecting control of HIV infection, and has the benefit of oral administration. Other PAH therapies including prostacyclin analogues, PDE5 inhibitors and selective endothelin receptor antagonists have yet to be trialed in this setting.