Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation

Four patients with presently uncorrectable cyanotic cardiac anomalies underwent a new operation, "total cavopulmonary shunt operation" or "total right heart bypass operation." These anomalies included single ventricle, single atrium, common atrioventricular valve with or without regurgitation, pulmonary stenosis, and most important, absent inferior vena cava with azygos or hemiazygos continuation. All patients had had previous systemic-pulmonary shunts. The new operation consisted of end-to-side anastomosis between the superior vena cava with azygos or hemiazygos continuation and the confluent pulmonary artery, division or ligation of the pulmonary artery trunk, and replacement of the common atrioventricular valve when regurgitation was present. Thus total venous return, except for hepatocardiac venous and coronary sinus flow, drains directly into the pulmonary artery, bypassing the right atrium and ventricle. Three patients survived the operation, and two of the three patients are now alive 4 years, 8 months and 2 years, 6 months after the operation. The two long-term survivors are in significantly improved condition, both clinically and hemodynamically. We believe that this new operation is promising in the treatment of the otherwise uncorrectable complex cardiac anomalies associated with azygos or hemiazygos continuation of the inferior vena cava. A longer follow-up is certainly mandatory before final conclusions can be reached.