Objectives: To examine baseline characteristics and outcome, and to determine variables affecting survival in patients with pulmonary arterial hypertension (PAH) associated with HIV infection (PAH-HIV) in the modern era of highly-active antiretroviral therapy (HAART) and specific PAH therapy.
Design: Retrospective review of data from PAH-HIV patients without other associated risk factors for PAH, and comparison with previous series.
Methods: Data were reviewed for 77 consecutive patients treated at the French Reference Centre for Pulmonary Hypertension between October 2000 and January 2008. Results were expressed as median [1st-3rd quartile] values.
Results: At diagnosis of PAH, 81% patients were on HAART, 79% had a CD4+ count more than 200 cells/microl and 49% had undetectable HIV load. New York Heart Association functional class assessment was II (22%), III (69%), and IV (9%). Six-minute walk distance (6MWD) was 375 [288-421] m, and pulmonary vascular resistance was 689 [524-852] dyn s/cm(5). All patients received HAART irrespective of HIV disease stage. Specific PAH therapy was started in 50 patients and led to improvements in 6MWD and haemodynamic parameters. In patients who did not receive specific PAH therapy, 6MWD improved but haemodynamics did not change. Overall survival rate was 88% at 1 year and 72% at 3 years. On multivariate analysis, cardiac index more than 2.8 l/min per m(2) and CD4+ lymphocyte count more than 200 cells/microl were independent predictors of survival.
Conclusion: In patients with PAH-HIV, HAART seems unable to improve haemodynamic parameters. Prognosis in PAH-HIV is mainly related to CD4+ lymphocyte count and cardiac function.