Prognostic Factors for Survival in Human Immunodeficiency Virus–associated Pulmonary Arterial Hypertension

Abstract

We report a large monocentric case series of 82 patients with human immunodeficiency virus-associated pulmonary arterial hypertension (PAH). No germline mutations of the PPH1 gene (bone morphogenetic protein receptor-II) were found in any of the 19 patients tested. PAH was the direct cause of death in 72% of cases. Survival rates of the overall population at 1, 2, and 3 years were 73, 60, and 47%, respectively. Survival was significantly poorer in patients in New York Heart Association functional class III-IV at the time of diagnosis, as compared with those in functional class I-II with respective rates of 60, 45, and 28% versus 100, 90, 84% at 1, 2, and 3 years (p < 0.0001). Subsequently, we analyzed prognostic factors in patients in functional class III-IV. Univariate analysis indicated that CD4 lymphocyte count of more than 212 cells mm(-3), the use of combination antiretroviral therapy (CART), and epoprostenol infusion were related with a better survival. On multivariate analysis only CD4 lymphocyte count was an independent predictor of survival, presumably because CART and epoprostenol infusion were strongly linked in our patient population. These results suggest that patients with severe human immunodeficiency virus-associated PAH should be considered for long-term epoprostenol infusion in association with CART.

Guidelines for the diagnosis and treatment of pulmonary hypertension

“…In a single-centre study, a better outcome was associated with CD4 count .212 cells?mL -1 , highly active antiretroviral therapy and epoprostenol therapy [122].…”

“…PAH is an independent risk factor for death in HIV-infected patients, with a 3-yr survival rate as low as 21% in the most advanced cases (WHO-FC III/IV), compared with 84% in mildly symptomatic patients [122]. In a single-centre study, a better outcome was associated with CD4 count .212 cells?mL -1 , highly active antiretroviral therapy and epoprostenol therapy [122].…”

“…[49]. More than 80% are well controlled on highly active antiretroviral therapy, and CD4 count does not appear to be a risk factor for PAH [49,122].…”

Human Immunodeficiency VirusnefSignature Sequences Are Associated with Pulmonary Hypertension

“…There is an increased mortality in HIV-PH subjects compared to normotensive HIV + individuals. 8 Unfortunately, many of the noninfectious complications of HIV infection, including PH, present with nonspecific symptoms and may be misdiagnosed.…”

“…Although mutations in the bone morphogenetic protein receptor-II have been identified in heritable PH, these mutations are not more prevalent in HIV-infected individuals. 8 HIV can be recovered from the lungs of humans and experimentally infected animals. [9][10][11] Hence, pulmonary vascular disease may be a consequence of the chronic exposure to viral proteins in the lung that may in turn drive the immune response.…”