Importance of coronary artery anomalies in operations for congenital heart disease.

A congenital coronary artery anomaly influenced operative management of 21 children (34 operations) during a recent 8-year interval. This group represented 1.3% of cardiothoracic operations performed. Survivors included 5 of 10 children having correction of the left coronary artery from the pulmonary artery and 11 children having correction of intracardiac defects associated with major coronary arteries crossing the right ventricular outflow tract. Two children survived primary repair of injured major coronary arteries. Isolated coronary artery anatomy must be considered during management of intracardiac defects. Intraoperative injury to a major coronary artery requires meticulous repair.

Extracardiac atrial pedicle conduit repair of partial anomalous pulmonary venous connection to the superior vena cava in children.

Between June, 1982, and July, 1983, 6 children with partial anomalous pulmonary venous connection to the middle or high segment of the superior vena cava (SVC) underwent repair of the anomaly by division of the SVC proximal to the site of entry of the anomalous pulmonary veins. Continuity between the cephalad end of the SVC and the right atrium was established by direct anastomosis to the right atrial (RA) appendage or by creation of a pedicle conduit of RA appendage, RA free wall, and pericardium. The anomalous pulmonary veins remained in situ on the lower segment of SVC, blood being directed to the left atrium through an atrial septal defect by a pericardial patch placed within the right atrium well away from the sinoatrial node, anomalous pulmonary veins, and cavoatrial junction. All children have survived, remain in normal sinus rhythm, and have no evidence of vena caval or pulmonary venous obstruction. Follow-up cardiac catheterizations, angiocardiograms, and Holter recordings support the efficacy of this technique as an alternative in the management of anomalous pulmonary veins joining the SVC well above the cavoatrial junction.

Individualized surgical management of complete atrioventricular canal.

In a 5 year interval 46 children with complete atrioventricular canal (CAVC) required 51 operations. Thirty-two children underwent correction; nine received surgical palliation. The ages of the children undergoing correction ranged from 4 months to 14.6 years (mean 4.2 years); eight were less than 1 year old. Weights ranged from 4.1 kg to 39 kg (mean 13.5 kg); 15 weighted less than 10 kg. Ten had undergone previous palliation (seven by pulmonary artery band; three by shunt). There were no early deaths and two late deaths. One infant required mitral replacement at correction; two required subsequent mitral replacement. The ages of the children undergoing palliation ranged from 8 days to 1.34 years (mean 5.8 months); 16 were less than 1 year old. Weights ranged from 2.5 kg to 8.5 kg (mean 4.4 kg); 14 weighted less than 5 kg. Operations included pulmonary artery banding in 14, shunt creation in four, and pericardial enlargement of the right ventricular outflow tract in one. One death occurred 5 days after pulmonary artery banding in an infant with unrecognized coarctation. One late death occurred several months after the creation of a second shunt in a child with severe tetralogy of Fallot and hypoplastic pulmonary arteries. Forty-two (91%) of these children were alive at the time of this review. The outcome in these 46 patients supports individualized choice of initial operation (palliation versus correction) based upon clinical condition, weight, and associated anomalies. The pulmonary artery is banded in infants less than 4 to 5 kg; larger infants and children undergo correction primarily.