HIV-Related pulmonary hypertension - Analytic review of 131 cases

Objective: To report two new cases of HIV-related pulmonary hypertension an d to review and analyze the existing reports on the subject. Method: Two new cases of HIV-related pulmonary hypertension are described, and the cases, case series, and related articles on the subject in all lang uages were identified through a comprehensive MEDLINE search. Results: Among the 131 reviewed cases, 54% were male, and the age range was 2 to 56 years (mean, 33 years). The interval between the diagnosis of HIV disease and the diagnosis of pulmonary hypertension was 33 months. In 82% o f cases, pulmonary hypertension was related solely to HIV infection. Presen ting symptoms were progressive shortness of breath (85%), pedal edema (30%) , nonproductive cough (19%), fatigue (13%), syncope or near-syncope (12%), and chest pain (7%), The mean (+/- SD) pulmonary arterial systolic BP was 6 7 +/- 18 mm Hg (n = 116), and diastolic BP was 40 +/- 11 mm Hg (n = 39), Pu lmonary vascular resistance was 983 +/- 420 dyne.s.cm(-5) (n = 29), Chest r adiographs demonstrated cardiomegaly (72%) and pulmonary artery prominence (71%). Right ventricular hypertrophy was the most common electrocardiograph ic finding (67%), Dilatation of the right heart chambers was the most commo n echocardiographic finding (98%). Plexogenic pulmonary arteriopathy was th e most common histopathology (78%), Pulmonary function tests demonstrated m ild restrictive patterns with variably reduced diffusing capacities, The re sponses to vasodilator agents and antiretroviral therapy was variable. Sixt y-six patients died during a median follow-up period of 8 months. The media n length of time from diagnosis to death was 6 months. Conclusion: HIV infection is an independent risk factor for the development of pulmonary hypertension. The appearance of unexplained cardiopulmonary s ymptoms in HIV-infected individuals should suggest pulmonary hypertension.