Left atrioventricular valve atresia: clinical management.

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Abstract
Children (40) with left atrioventricular valve (LAV) atresia without aortic stenosis or atresia were reviewed. Thirty-one had mitral atresia and 9 had left tricuspid atresia. Eleven had associated pulmonary outflow tract obstruction. Twenty-two (55%) underwent cardiac catheterization and 12 had more than 1 study. Left atrial pressure was normal in 7 infants younger than 2 wk of age. Twelve patients had an elevated mean left atrial pressure (16-38 mm Hg), including 2 who had normal left atrial pressures at an earlier study. Apparently an initially adequate interatrial opening has the potential to become restrictive. Balloon atrial septostomy is recommended in all patients with LAV atresia who are less than 1 mo. of age at initial study. Thereafter, serial cardiac catheterizations should be performed to assess changing hemodynamics. Twenty-three patients (56%) had palliative operations to decrease left atrial hypertension (atrial septectomy) and/or optimize pulmonary blood flow (pulmonary artery banding, ductus ligation or systemic-to-pulmonary anastomosis). Nine survivors (23%) were followed from 13 mo. to 23 yr (median 7 yr). Of these, 8 had at least 1 surgical procedure. Prognosis was best in patients with mild to moderate pulmonic stenosis and worst in cases with pulmonic atresia. Palliative surgery may improve survival in selected patients with LAV atresia.