Twenty-six patients in infancy and early childhood with severe pulmonary valve stenosis and intact ventricular septum are reviewed. They were selected from a larger series of 112 patients with pulmonary stenosis of any degree, on account of early onset of symptoms and the severity of the stenosis proven by cardiac catheterization and angiocardiography, at operation or at necropsy. Our criteria for severity in this series were: presence of symptoms within the first two years of life; right ventricular and right atrial hypertrophy on electrocardiography; and right ventricular pressure equal to or higher than systemic blood pressure. The warning signs prompting valvotomy are deterioration of the following features: cyanosis and dyspnoea; congestive cardiac failure; tricuspid incompetence; cardiac enlargement and pulmonary oligaemia on radiograph; and right ventricular and right atrial hypertrophy on electrocardiography. The lives of 13 patients were saved by timely valvotomy. These patients are all well six months to six years after operation. Five patients died before any operation could be performed. Eight patients died within 48 hours of operation. Had some of these patients been operated on earlier the evidence indicates that they would have had a better prognosis. Therefore the importance of early recognition, prompt treatment, and emergency valvotomy, if necessary, is emphasized.
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