ESSENTIAL PULMONARY HYPERTENSION: A REPORT OF CLINICAL-PHYSIOLOGIC STUDIES IN THREE PATIENTS, WITH DEATH FOLLOWING CATHETERIZATION OF THE HEART

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Abstract
Two of the patients were children, one was an adult. Clinical findings showed dyspnea and syncope on exertion. Physical findings included heart murmurs and absence of cyanosis. Other findings were right ventricular hypertrophy, cardiac enlargement and a prominent pulmonary artery segment. Catheterization findings revealed marked increase in pulmonary artery pressure and the pulmonary vascular resistance. Pathological findings were marked narrowing of the pulmonary arterioles through medial and intimal hypertrophy and arteriosclerosis of the pulmonary arteries.