Excellent midterm outcome of extracardiac conduit total Cavopulmonary connection: Results of 126 cases

Background. Extracardiac conduit total cavopulmonary connection has shown good early results; however, its long-term outcome has yet to be reported. Methods. Of 282 patients who underwent extracardiac conduit total cavopulmonary connection since 1994, 126 patients who have been followed up for more than 5 years were included in this retrospective study. Actuarial survival rate, incidence of late complications, hemodynamic variables, and results of exercise tolerance test were reviewed. Results. Follow-up time was 96.4 +/- 23.0 months. There was 1 operative death and 6 late deaths. Actuarial survival rate was 95.2% and 93.6% at 5 and 10 years, respectively. Seven patients had late complications including new-onset supraventricular arrhythmia in 3, protein-losing enteropathy in 2, thromboembolism in 1, and bleeding complication in 1. Seven patients underwent reoperation not related to the conduit. Freedom from Fontan-related events was 88.8% at 5 years and 84.3% at 10 years. Late cardiac catheterization in 119 survivors showed central venous pressure of 9.9 +/- 2.9 mm Hg, cardiac index of 3.6 +/- 0.8 L center dot min(-1) center dot m(-2) and arterial oxygen saturation of 94.5 +/- 2.3%. No patient showed conduit stenosis. Plasma concentration of atrial and brain natriuretic peptide (pg/mL) were 28.9 +/- 20.0 and 25.8 +/- 44.5. Exercise test performed in 101 patients showed endurance time of 75.7 +/- 12.9% of normal value, peak heart rate of 92.3 +/- 14.4% of normal, and peak oxygen consumption of 90.0 +/- 20.0% of normal. The latest echocardiogram showed ejection fraction of 60.4 +/- 11.7%. Three patients had pacemaker rhythm, 1 had junctional rhythm, and 115 patients had sinus rhythm. Conclusions. Midterm outcome of extracardiac conduit total cavopulmonary connection was satisfactory with low incidence of late mortality and morbidity, and excellent hemodynamic state.