Objectives: HIV-related pulmonary arterial hypertension (PAH) is a rare entity but is associated with significant morbidity and mortality. The literature describing the outcomes of therapy for this disease is limited to case series and cohort studies. The objective of this study was to systematically review and synthesize the literature on HIV-related PAH.
Methods: MEDLINE, EMBASE, PapersFirst, the Cochrane collaboration and the Cochrane Register of controlled trials were searched with pre-defined search terms. Randomized controlled trials, observational cohort studies, case-control studies and case reports were considered for inclusion in the qualitative analysis.
Results: A total of 180 case reports of PAH in HIV-infected patients were identified. Twenty-six were excluded and thus 154 case reports were included in the qualitative analysis. Thirteen cohort, one case series and two case-control studies were also identified and included in the review. The average baseline CD4 count at the time of diagnosis of PAH was 352 ± 304 cells/μL. The average time from diagnosis of HIV infection to diagnosis of PAH was 4.3 ± 4.0 years. Predominant chest X-ray findings included cardiomegaly (80%) and pulmonary arterial enlargement (75%). Highly active antiretroviral therapy, bosentan, and prostaglandin therapy have all been reported to be beneficial in improving haemodynamic and functional status in HIV-related PAH.
Conclusion: HIV-related PAH is a rare entity with clinical, laboratory, imaging and pathological manifestations similar to those of idiopathic PAH. The evidence for various treatments is limited to cohort, case series and case-control studies. Randomized controlled trials are needed to properly assess the utility of these therapies in the treatment of HIV-related PAH.
© 2010 British HIV Association.