Atresia of left atrioventricular connection. Surgical considerations.

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Abstract
Patients (17) diagnosed in life as haivng no left atrioventricular connection (14 patients) or an imperforate left atrioventricular valve (3 patients) were studied. Ten patients presented at < 3 mo. with tachypnea, mild cyanosis and heart failure. Seven patients, including 2 with moderate and 1 with severe pulmonary outflow tract obstruction, presented at a mean age of 45 mo. (range 6 mo. to 16 yr). Six patients had a non-restrictive interatrial communication when first catheterized. In the remainder the mean onteratrial gradient was 14 mm Hg. Excluding patients with pulmonary outflow tract obstruction the mean pulmonary vascular resistance was 6.7 U/m2. Patients were managed by balloon atrial septostomy (4), atrial septectomy (9), pulmonary artery banding (9), systemic pulmonary anastomoses (1) and a modified Fontan''s procedure (1). There were 5 hospital deaths. Two followed pulmonary artery banding and 3 followed atrial septectomy. The mean length of follow-up in the surviving patients was 3 1/2 yr (range 5 mo. to 15 yr). The 6 patients who had an atrial septectomy, and 1 patient who had a modified Fontan''s procedure, were well palliated with minimal reduction in exercise tolerance and mild cyanosis at rest. Three patients, one with an atrial septostomy, 1 who had pulmonary artery banding, and 1 who had no surgical procedure had evidence of pulmonary vasular disease. A further patient who had a balloon septostomy had ECG evidence of left atrial hypertrophy. Atrial septectomy plus or minus pulmonary artery binding provides good palliation and prevents the development of pulmonary vascular disease. A modified Fontan''s procedure should be considered as an alternative or subsequent procedure in patients with suitable anatomy.