Chest
Volume 100, Issue 5, November 1991, Pages 1268-1271
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Clinical Investigations
Primary Pulmonary Hypertension in HIV Infection

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A prospective evaluation of 74 human immunodeficiency virus (HIV)-infected patients with cardiopulmonary complaints revealed six patients (8.1 percent) with pulmonary hypertension with elevated right ventricular systolic over right artrial pressure of 58 ± 8 mm Hg (range, 49 to 66 mm Hg), as documented by Doppler echocardiography. A thromboembolic cause was excluded by normal lung perfusion scans. Electrocardiographic and roentgenographic features of pulmonary hypertension were present in five patients. Two patients died three and nine months after diagnosis of pulmonary hypertension. Autopsy revealed plexogenic pulmonary arteriopathy in both. The observation of six patients with primary pulmonary hypertension (PPH) in a cohort of 1,200 HIV-infected subjects corresponding to an incidence of 0.5 percent is striking and suggests a possible association of PPH with HIV infection.

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Patients and Methods

Over a nine-month period (January to September 1990), we prospectively examined by bronchoscopy and/or echocardiography 74 consecutive HIV-infected patients for complaints due to cardiopulmonary impairment, ie, cough of ≥two weeks' duration, shortness of breath, and/or abnormal blood gas values, pulmonary infiltrates, cardiac murmurs, fever >38.5°C, or positive blood cultures not explained by extrathoracic infections. This patient group is part of a cohort of 1,200 HIV-positive subjects

Results

The six patients with a mean age of 30 ± 5 years (range, 24 to 37 years) include five female IV drug users and one male homosexual. The presenting symptoms lasting between one to six months consisted of pedal edema (n = 5) and/or dyspnea on exertion (n = 3) with syncope and palpitations (n = 1). Three patients suffered from symptomatic HIV disease (CDC IV) and in three the CD4 T lymphocytes in the peripheral blood were markedly decreased (Table 1). Pulmonary hypertension with RVSP of 58 ± 8 mm

Case Reports

This 37-year-old woman (case 2) had a history of IV drug abuse from 1974 to 1987. She presented in January 1990 with spastic paraparesis due to suspected HIV-associated myelopathy, thrombocytopenia, leukopenia, and mild pedal edema. Electrocardiography showed signs of right ventricular hypertrophy. Whereas a chest roentgenogram was normal one year before (Fig 1), cardiomegaly with prominence of the main pulmonary artery was visible at the time of hospital admission (Fig 1). The lung fields were

Discussion

Primary pulmonary hypertension is a rare disorder. Only 187 cases have been reported to the National Heart, Lung, and Blood Institute, Bethesda, Md, during a period of four years.5 The diagnosis is found in less than 0.25 to 1.1 percent of unselected right heart catheterizations.15 At the inpatient and outpatient clinic of our referring university hospital with a population area of more than one million, we see not more than five cases each year. Therefore, the observation of PPH in six

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Reprint requests: Dr. Speich, Department of Internal Medicine, University Hospital, 8091 Zurich, Switzerland

Manuscript received October 29; revision accepted March 8.

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