Twenty years of data from the Spanish pulmonary arterial hypertension (PAH) Registry revealed similarities and differences in characteristics, treatment patterns, and prognosis of patients with PAH from different etiologies, as published in the Journal of Internal Medicine.

Patients with HIV-associated PAH accounted for nearly 7% of the entire Spanish PAH population included in the database. These patients (n=132) are mostly young males, while patients with idiopathic or familial PAH (IPAH/FPAH, n=739) are predominantly older women. The most common mode of HIV transmission was parenteral drug use.

Short-term prognosis and overall survival were similar between age- and sex-matched HIV-PAH and IPAH/FPAH patients. In addition, both 1- and 3-year survival rates remained similar over time. However, patients with HIV-PAH showed worse pulmonary functions but better functional states than patients with PAH/FPAH.

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“The greater delay in IPAH/FPAH diagnosis is likely to be the reason for the worse functional status of patients with IPAH/FPAH, even after matching both populations by age and sex,” the authors said.

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PAH clinical presentation and hemodynamics were also similar between groups, albeit chest pain was more frequently reported by patients with HIV-PAH than patients with PAH/FPAH (24.2% vs 16.8%, P =.048). The difference in the frequency of chest pain between groups decreased when the populations were matched by age and sex.

Heart failure was the main cause of death in patients with HIV-PAH, but it was even more frequent in patients with IPAH/FPAH (24% vs 48%). Cancer was the cause of death in 17.1% of patients with HIV-PAH and 1.3% of patients with IPAH/FPAH.

Oral monotherapy was the preferred first-line treatment in both groups. Dual oral combination therapy was used in a higher proportion of patients with IPAH/FPAH in comparison to patients with HIV-PAH. Also, 88.4% of the patients with HIV-PAH with HIV treatment information available received antiretrovirals.


Lázaro SM, Rodríguez‐Padial L, Soto Abánades C, et al. Management and prognosis of HIV‐associated pulmonary arterial hypertension: 20 years of evidence from the REHAP registry. J Intern Med. Published online February 20, 2022. doi:10.1111/joim.13468