Pulmonary stenosis in the first year of life.

Open Access
Publisher Website
Google Scholar
Abstract
Severe pulmonary stenosis with an intact ventricular septum in the 1st yr. of life may be an extremely dangerous lesion; its recognition and prompt surgical therapy is most rewarding. Between Jan. 1956 and Dec. 1965 at the Toronto Hospital for Sick Children, 26 pulmonary valvotomies were performed in infants of less than 1 yr. of age for pulmonary stenosis with intact ventricular septum, with 1 fatal outcome (4% mortality). Of the 26 patients, 23 had some degree of cyanosis and. 11 of them had right heart failure; all had a systolic murmur. Five neonates had ecg evidence of pure left ventricular hypertrophy. Right atrial hypertrophy was present in 15 patients. Cardiomegaly of some degree was present in 21 infants and pulmonary vascularity was generally reduced. Post-stenotic dilatation of the pulmonary artery, so frequently present in older children, was minimal or absent. Pre-operative right heart catheterization in 22 infants revealed right ventricular pressures ranging between 115 and 186 mm Hg, except in 2 cases where it was 88 and 95 mm Hg, respectively. Selective cine-angiocardiography was done in 17 patients and is the best method of obtaining an accurate diagnosis, because of the difficulty in catheterizing the pulmonary artery through the excessively narrow valve orifice. Transarterial pulmonary valvotomy under direct vision with inflow occlusion and normothermia was uniformly successful regardless of the age, size, or pre-operative condition of the patient. Early in the series mild hypothermia was employed but has now been abandoned because of the difficulty of resuscitating the cold heart. Hypothermia is not only unnecessary but possibly dangerous.