HIV- associated pulmonary arterial hypertension

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. HIV infection is an established risk factor for the development of PAH. In comparison with the incidence of idiopathic PAH in the general population (1-2 per million), HIV-infected patients have a 2500-fold risk of developing PAH. The presence of PAH is an independent risk factor for mortality in patients with HIV infection, and in most cases death is causally related to PAH rather than to other complications of HIV infection. HIV-associated PAH (HIV-PAH) occurs at all stages of disease and does not seem to be related to the stage of HIV infection, the degree of immune deficiency or cD4 T-lymphocyte count. since highly effective therapies for PAH are now available, allowing an amelioration of symptoms and a better prognosis, clinicians should be aware that the appearance and progression of shortness of breath and other cardiopulmonary symptoms in HIV-infected patients may be suggestive of HIV-PAH. This paper will focus on HIV-PAH with special considerations to epidemiology, pathogenesis, clinical features and diagnosis, and treatment.