Clinical study
Primary pulmonary hypertension: I. Clinical and hemodynamic study

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Abstract

The concept of primary pulmonary hypertension is defined and the clinical and hemodynamic features in three patients with this disease are presented. Such cases present a clinical syndrome that has been previously described under various names, e.g., “primary pulmonary vascular sclerosis,” “right ventricular hypertrophy of obscure origin” and “idiopathic pulmonary hypertension.”

The salient clinical features in this syndrome are exertional weakness and dyspnea in patients who subsequently develop right heart failure without antecedent cardiac or pulmonary disease. Effort syncope and angina are most significant when present. Sudden death is not infrequent. The pertinent physical findings are a normal systemic blood pressure, clear lungs, an accentuated pulmonic second sound and the variability or absence of heart murmurs. Venous distention and hepatomegaly occur unassociated with peripheral edema or ascites except late in the course of the disease. Cyanosis, when encountered, is a terminal feature unless the disease is complicated by a patent foramen ovale. This syndrome has been seen in both sexes at all ages but is most common between the ages of twenty and forty years.

The electrocardiographic tracings are consistent with right ventricular hypertrophy. The characteristic x-ray findings are (1) right ventricular enlargement, (2) a bulging pulmonary artery segment, (3) prominent hilar vessels and (4) normal or diminished intrapulmonary vascular markings.

Marked right ventricular hypertrophy is a constant pathologic finding in primary pulmonary hypertension but the nature, severity and distribution of the pulmonary vascular changes proximal to the capillary bed show considerable variation. In some cases no significant pulmonary vascular changes are noted.

Pulmonary function studies were performed in two of the patients and ruled out intrinsic lung disease. Hemodynamic studies, utilizing the technic of right heart catheterization, were performed in the three patients presented in this series. All had greatly elevated pulmonary artery pressures, elevated right ventricular end diastolic pressures, diminished cardiac outputs, increased arteriovenous oxygen differences and normal arterial blood oxygen saturations. The blood volumes and hematocrits were normal in two of the patients and slightly elevated in the third. One patient had no clinical evidence of right heart failure and none had edema.

An attempt was made to correlate the hemodynamics at rest and during exercise with some of the clinical findings. A seven- to ninefold increase in pulmonary resistance was calculated at rest. Physiologic studies and postmortem findings were presented to support the view that the locus of the increased resistance is in the small pulmonary arteries.

Isolated overactivity of the sympathetic nervous system was suggested by the pronounced effect of priscoline, an adrenolytic and sympatholytic agent, in lowering the pulmonary artery blood pressure. On the basis of the material presented the problem of sympathectomy in primary pulmonary hypertension was raised.

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    This investigation was supported in part by a research grant from the National Heart Institute of the National Institutes of Health, Public Health Service.

    1

    From the Medical Service, Maimonides Hospital of Brooklyn and the Department of Medicine, State University of New York at New York City, College of Medicine.

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