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The spontaneous occurrence and teratogenic production of atrial septal defect in A/J mice and ventricular septal defect in C57BL/6J mice lead to the proposal that these strains may represent animal homologies of these congenital heart diseases in human beings. Such models permit investigation into the mechanisms by which nongenetic factors cause genetically predisposed individuals to reach a particular threshold of developmental abnormality and develop a specific type of cardiac malformation.

A definitive program for the medical and surgical management of anomalous left coronary artery arising from the pulmonary artery is proposed based on experience with 10 infants and children with this diagnosis seen at Texas Children's Hospital. The 10 patients have been evaluated by history, physical examination, electrocardiography, vectorcardiography, cardiac catheterization, and selective angiocardiography. Although the mortality is reported to be 80% in patients with evidence of infarction, symptoms in infancy, and absence of a systolic-diastolic murmur, a more encouraging mortality of only 20% was found in this study. Medical management of the congestive heart failure is the only treatment so far offered to three patients. Three early patients have had ligation of the left coronary artery; two of these patients died postoperatively. Four recent patients have had definitive surgical correction of the anomaly by separation of the coronary artery from the pulmonary artery and anastomosis to the aorta. All of these patients have survived, and three have postoperative angiographic demonstration of a functioning two-coronary artery system. Because definitive surgical therapy is now possible, the authors feel that medical management until an age suitable for graft anastomosis of the anomalous vessel to the aorta is the treatment of choice with the lowest mortality in infancy and childhood and the most satisfactory long-term outlook.

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