PRIMARY PULMONARY HYPERTENSION IN CHILDHOOD

Abstract

Four cases of primary pulmonary hypertension are described in children, aged 4 to 9 years. Clinical, electrocardiographic, radiographic, and cardiac catheterization findings are similar in most respects to the cases usually described in adults. The group is distinct from another group of infants who died with right ventricular hypertrophy and right ventricular failure that remained unexplained after autopsy. A lysergic acid derivative with anti-serotonin properties (UML 491) in modest dosage failed to reduce the pulmonary vascular resistance in two cases. Clinical and ancillary evidence suggested that the disease was an acquired one. Examination of the main trunks of the pulmonary arteries was carried out in the 3 fatal cases, but unequivocal support for the acquired nature of the disease was not obtained. Thrombosis in small arteries was the dominating feature in the lungs. No certain cause for the pathological changes was discovered, nor was it suggested that the cause was the same in each case. Three of the four cases presented for examination within a period of 2 months.