title: Pulmonary arterial hypertension related to human immunodeficiency virus infection: A case series.
abstract: OBJECTIVE
  To present 18 new cases of human immunodeficiency virus (HIV)-related pulmonary arterial hypertension (PAH) with presenting features, treatment options and follow-up data.
  
  
  METHODS
  This is a single-centre, retrospective, observational study that used prospectively collected data, conducted during a 14-year period on HIV-related PAH patients who were referred to a pulmonary hypertension unit. All patients infected with HIV were consecutively admitted for an initial evaluation of PAH during the study period and included in our study. Right heart catheterisation was used for the diagnosis of PAH. Specific PAH treatment was started according to the physician's judgment and the recommendations for idiopathic PAH. The data collected included demographic characteristics, parameters related to both HIV infection and PAH and disease follow-up.
  
  
  RESULTS
  Eighteen patients were included. Intravenous drug use was the major risk factor for HIV infection. Risk factors for PAH, other than HIV infection, were present in 55.5% patients. The elapsed time between HIV infection and PAH diagnoses was 12.2 ± 6.9 years. At PAH diagnosis, 94.1% patients had a CD4 cell count > 200 cells/μL. Highly active antiretroviral therapy (present in 47.1% patients) was associated with an accelerated onset of PAH. Survival rates were 93.8%, 92.9% and 85.7% at one, two and three years, respectively. Concerning specific therapy, 33.3% of the patients were started on a prostacyclin analogue, and the rest were on oral drugs, mainly phosphodiesterase-5 inhibitors. During the follow-up period, specific therapy was de-escalated to oral drugs in all of the living patients.
  
  
  CONCLUSIONS
  The survival rates of HIV-related PAH patients were higher, most likely due to new aggressive specific therapy. The majority of patients were on oral specific therapy and clinically stable. Moreover, sildenafil appears to be a safe therapy for less severe HIV-related PAH.
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Inês
authors: I
authors: Araújo
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Cristina
authors: C
authors: Enjuanes-Grau
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Carmen Jimenez
authors: CJ
authors: Lopez-Guarch
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Dariusz
authors: D
authors: Narankiewicz
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Maria J
authors: MJ
authors: Ruiz-Cano
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Teresa
authors: T
authors: Velazquez-Martin
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Juan
authors: J
authors: Delgado
authors: Inês Araújo, 3 Medicine Department, São Francisco Xavier Hospital, CHLO, 1449-005 Lisboa, Portugal.
authors: Pilar
authors: P
authors: Escribano
keywords: Human immunodeficiency virus infection; Pulmonary arterial hypertension; Treatment