HUMAN-IMMUNODEFICIENCY-VIRUS INFECTION AND PULMONARY-HYPERTENSION - 2NEW CASES AND A REVIEW OF 86 REPORTED CASES

In this article, we describe pulmonary hypertension in two men (31 and 43 years of age) with human immunodeficiency virus (HIV) infection wh o were examined at Mayo Clinic Rochester, Among 88 reported cases (inc luding the two current ones) of HIV-or acquired immunodeficiency syndr ome (AIDS)-associated pulmonary hypertension, 61% were male; the age r ange was 2 to 56 years (mean, 32). Dyspnea was the usual initial sympt om. Of the 74 patients in whom pulmonary artery pressure was' recorded or calculated by echocardiography, systolic pressures ranged from 49 to 118 mm Hg (mean, 68). Of the 33 cases in which lung tissue was eval uated microscopically, 28 (85%) were of the plexogenic variant of pulm onary arterial hypertension, Of the other five cases examined histolog ically, three consisted of thrombotic pulmonary arteriopathy (one was due to recurrent thromboembolism, and the other two were due to in sit u thrombosis), and two were of pulmonary venoocclusive disease. No cor relation existed between either CD4 counts or a history of pulmonary i nfections and the development of pulmonary hypertension, In 15 of the 88 patients (17%), confounding factors for hypertensive pulmonary vasc ular disease were present, including coexisting liver disease in 13 an d coagulation abnormalities in 2, In 83% of the patients, the developm ent of pulmonary hypertension seems to have been related primarily to the chronic HIV infection, Pulmonary hypertension was more rapidly pro gressive in patients with HIV or AIDS than in those with primary pulmo nary hypertension; the reported time intervals between onset of sympto ms and diagnosis were 6 months and 30 months, respectively, The 1-year survival rate for patients with HIV and pulmonary hypertension was 51 %, based on the follow-up data compiled from the 63 patients in whom i t was described; this compares with a 1-year survival rate of 68% for patients with primary pulmonary hypertension, Death was considered a d irect consequence of pulmonary hypertension in 29 (76%) of the 38 fata l cases.