Pulmonary arterial hypertension (PAH) is a rare but severe disease that results from chronic obstruction of small pulmonary arteries, leading to right ventricular failure and, ultimately, death. One established risk factor for the development of PAH is HIV infection. The presence of PAH is an independent risk factor for mortality in HIV-infected patients. This article will focus on HIV-associated PAH (HIV-PAH) with special considerations to the available treatments. With the approval of the soluble guanylate cyclase stimulator riociguat, a new drug class has become available in addition to the already existing prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. Guidelines for the treatment of idiopathic PAH and guidelines for antiretroviral therapy should be followed for the treatment of HIV-PAH.
Keywords: HIV; Pulmonary arterial hypertension; antiretroviral therapy; endothelin receptor antagonists; phosphodiesterase type 5 inhibitors; prostanoids; riociguat; therapy.