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Summary Between June 1988 and December 1992, six patients with dominant left and rudimentary right ventricles underwent orthoterminal correction with a modified Fontan operation in which the atriums were neoseptated using a flap constructed from the right atrial wali. Four patients had anomalous systemic venous connections. The operations were performed under direct caval cannulation, standard cardiopulmonary bypass, moderate hypothermia and cardioplegic arrest. After opening the right atrium with a longitudinal incision, the atrial septum was completely resected and the coronary sinus was cut back. The upper wall of the right atrial incision was brought down in such a way that the pulmonary venous atrium was drained into the dominant ventricle via the right-sided or common atrioventricular valve. This was followed by direct connection of the right atrium with its anterior wall reconstructed with pericardium to the pulmonary arteries. In one patient, a left superior caval vein draining to the left atrium was divided and anastomosed to left pulmonary artery. One patient died on the 12th postoperative day with pulmonary infection and sepsis, though he had no hemodynamic problem, and another died on the sixth day due to high pulmonary vascular resistance. The remaining four patients are progressing well at a mean of 23.4 months postoperatively with functional capacity of NYHA I-IT and sinus rhythm. Echocardiographic and angiocardiographic examinations during follow-up showed unobstructed pulmonary and systemic venous pathways in all. This modification of the Fontan operation seems a good alternative technique which creates a contractile left atrium with large enough dimensions and an unobstructed pathway for pulmonary venous flow.
Between January 1989 and March 1993, total correction was performed in 99 patients with tetralogy of Fallot without submitting them to prior cardiac catheterization. The age of the patients ranged from 1.33 to 18 years (mean 5.33±3.77). After complete echocardiographic examination, the diameters of the right and left pulmonary arteries at the prebranching point and the descending thoracic aorta at the diaphragm were measured by cross-sectional echocardiography and the McGoon ratio was calculated. Total correction was performed in all patients with a McGoon ratio greater than 1.7. In none of the patients were the sizes of the pulmonary artery measured by echocardiography smaller than the measurements obtained during surgery. Transannular patching was performed in 76 patients. A conduit from the right ventricle to the pulmonary arteries was constructed in two patients with coronary arterial anomalies. Postrepair right ventricular to left ventricular systolic pressure ratios were between 0.25 and 0.85 (mean 0.54±0.13). There were two hospital deaths, neither being related to the diagnostic method used nor the criteria for surgery. We conclude that the diagnosis of tetralogy of Fallot together with measurements of pulmonary arteries and descending thoracic aorta can safely and reliably be achieved echocardiographically. The McGoon ratio can be adapted to echocardiography and total correction can be performed successfully based on echocardiographic examination.
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