Management and prognosis of HIV‐associated pulmonary arterial hypertension: 20 Years of evidence from the REHAP registry.

Background: Pulmonary arterial hypertension (PAH) is an independent predictor of death in patients with human immunodeficiency virus (HIV) infection. HIV is the leading cause of PAH (HIV‐PAH) worldwide. Aims: We described the characteristics, treatment patterns, and prognosis of a cohort of HIV‐PAH patients and compared them with those of an equivalent cohort of patients with idiopathic/familial PAH (IPAH/FPAH). Methods: We retrospectively analysed and compared the demographic, clinical, and treatment data from patients with HIV‐PAH and those with IPAH/FPAH in the Spanish PAH registry (REHAP) from 1998 to 2018. The HIV‐PAH overall survival (OS) rate up to 5 years was compared to the age‐ and sex‐matched IPAH/FPAH population. Changes in treatment patterns in patients with HIV‐PAH after 2010 and their effects on OS were also analysed. Results: Compared to those with IPAH/FPAH (n = 739), patients with HIV‐PAH (n = 132) were younger, mainly men, and had a better functional status. The clinical presentation, haemodynamics, and respiratory function were similar between the groups. Parenteral drug use was the most common mode of HIV transmission. Approximately 11% of patients with HIV‐PAH did not receive PAH‐targeted therapy. The age‐ and sex‐adjusted 5‐year OS rate from diagnosis was 74.0% for patients with HIV‐PAH and 68.7% for those with IPAH (p < 0.159). During/after 2010, 23% of patients with IPAH/FPAH received upfront dual oral combination, while oral monotherapy remained the main first‐line treatment in patients with HIV‐PAH. The overall OS rate remained stable. Conclusions: Patients with HIV‐PAH were predominantly young men. The short‐term prognosis is similar to that of age‐ and sex‐matched patients with IPAH/FPAH, despite a better functional status. Oral monotherapy remains the preferred first‐line treatment in the current cohorts. [ABSTRACT FROM AUTHOR]