THE EXTRACARDIAC TOTAL CAVOPULMONARY CONNECTION FOR DEFINITIVE CONVERSION TO THE FONTAN CIRCULATION - SUMMARY OF EARLY EXPERIENCE AND RESULTS

Between July 1991 and March 1993, five children (ages 2 to 6 years) wi th complex congenital heart disease have undergone a new operation for conversion to the Fontan circulation. This procedure combines a bidir ectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carr y systemic venous return to the pulmonary arteries (PAs). The ELT was constructed so that the circumference consists of Gore-Tex (2/3) and l ateral epicardial atrial wall (1/3). The ELT can be performed with all varieties of single ventricle physiology, as in our patients with tri cuspid atresia (n = 3), dextrocardia (n = 1), and situs inversus with levocardia (n = 1). PA reconstruction was required in four patients. A t follow-up from 1 to 20 months, all patients are in New York Heart As sociation Class I and in normal sinus rhythm. Postoperative catheteriz ation has revealed low PA pressures (less-than-or-equal-to 12 +/- 1 mm Hg) and angiography has shown excellent ELT function with brisk flow i nto the PAs bilaterally. All patients maintain an O2 saturation > 94% on room air. The advantages of this new extracardiac modification of F ontan's operation are: (1) aortic cross-clamping is not usually requir ed; (2) incorporation of lateral atrial wall in ELT allows for growth while permitting construction of a fenestration or adjustable atrial s eptal defect in high risk patients; (3) absence of atriotomy and intra atrial suture lines may decrease late risk of arrhythmias; (4) early o r late baffle leaks cannot occur; (5) intraatrial obstruction from the baffle cannot occur; (6) coronary sinus remains in low pressure atriu m; and (7) hydrodynamic benefits of the total cavopulmonary connection are preserved. We recommend this procedure for patients undergoing su rgical conversion to the Fontan circulation.