Objective: To report two new cases of HIV-related pulmonary hypertension and 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 languages 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% of cases, pulmonary hypertension was related solely to HIV infection. Presenting 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 67 +/- 18 mm Hg (n = 116), and diastolic BP was 40+/-11 mm Hg (n = 39). Pulmonary vascular resistance was 983+/-420 dyne. s. cm(-5) (n = 29). Chest radiographs demonstrated cardiomegaly (72%) and pulmonary artery prominence (71%). Right ventricular hypertrophy was the most common electrocardiographic finding (67%). Dilatation of the right heart chambers was the most common echocardiographic finding (98%). Plexogenic pulmonary arteriopathy was the most common histopathology (78%). Pulmonary function tests demonstrated mild restrictive patterns with variably reduced diffusing capacities. The responses to vasodilator agents and antiretroviral therapy was variable. Sixty-six patients died during a median follow-up period of 8 months. The median 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 symptoms in HIV-infected individuals should suggest pulmonary hypertension.