Midterm to Long-Term Outcome of Total Cavopulmonary Connection in High-Risk Adult Candidates

Background Adult patients who do not fulfill the classical Fontan criteria now undergo total cavopulmonary connection (TCPC). However, limited information is available on the results for high-risk adult TCPC. Methods Twenty-five consecutive adult patients (aged 16 years or more) who underwent TCPC were retrospectively reviewed. The mean age at operation was 27 ± 9 years (range, 16 to 52). The following items were considered as the potential risk factors according to previous reports: (1) aged more than 30 years (7 of 25); (2) heterotaxy (9 of 25); (3) systemic ventricular ejection fraction less than 50% (6 of 25); (4) atrioventricular valve regurgitation moderate or greater (6 of 25); (5) pulmonary arterial index less than 200 (7 of 25); (6) mean pulmonary arterial pressure 15 mm Hg or greater (3 of 25); (7) pulmonary arterial resistance 2.0 wood units or greater (11 of 25); (8) arrhythmias (13 of 25); (9) protein-losing enteropathy (3 of 25); (10) New York Heart Association (NYHA) functional class III or greater (9 of 25); (11) previous Fontan procedure (10 of 25); (12) systemic ventricular outflow obstruction (1 of 25); and (13) end-diastolic pressure of the systemic ventricle 11 mm Hg or higher (4 of 25). Results The mean follow-up period was 57 ± 45 months (range, 0 to 154). All patients had at least 2 risk factors (range, 2 to 8). There was 1 early death and 2 late deaths. Comparing the late survivors and nonsurvivors, no statistical significance was identified in the above risk factors. However, the patients with 6 or more risk factors had a significantly higher mortality rate than patients with fewer than 6 risk risk factors ( p < 0.01). Age ( p = 0.08), NYHA class ( p = 0.13), and protein-losing enteropathy ( p = 0.08) may be risk factors for late death. Conclusions The majority of the adult TCPC candidates tolerated the TCPC procedure in the early postoperative period. However, the accumulation of risk factors influences late mortality.