AIM: 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 hy-pertension 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.CONCLUSION: 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 HIVrelated PAH. AIM: 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-center, retrospective, observational study that used prospectively collected data, conducting during a 14-year period on HIV-related PAH patients who were referred to a pulmonary hy-pertension 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 catheterization 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 the the major risk factor for HIV infection. Risk factors for PAH, other than HIV infection, we At elapsed time of 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 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. CONCLUSION: 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 HIVrelated PAH.