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 · min^-1 · 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.