Right ventricular morphology is a known risk factor for death in patients with univentricular hearts (UVH), especially before partial cavopulmonary connection. This study investigates the influence of anatomic and procedure related factors on mid-term outcome after extracardiac total cavopulmonary connection (TCPC).

Retrospective single-center analysis of all patients with UVH undergoing extracardiac TCPC from 2000 to 2005. Patient and procedure related variables were analyzed.

100 patients underwent extracardiac TCPC in the study period. 69% had a dominant left ventricle, 23% had a dominant right ventricle and 8% had biventricular anatomy. Median age at intervention was 6.1years (1.1–29.4). At the moment of surgery, 9% were in NYHA class>2 or had preoperative univentricular dysfunction. 6% had significant atrioventricular valve (AVV) regurgitation. Median preoperative mean pulmonary pressure was 12mmHg (6–21). Early mortality was 4% and global mortality was 8%. Anatomic, functional, perioperative and postoperative parameters did not impact mortality. Only preoperative NYHA class>2 was significantly associated to higher global mortality in univariate analysis. Freedom from death/transplantation was 92.6% (95% CI±0.05) at 5years and 91.4% (95% CI±0.06) at 10years after TCPC. Ventricular morphology did not influence global mortality (log rank P=0.22) or mid-term morbidity (thrombosis, protein loosing enteropathy, arrhythmia; log rank P=0.76). Median follow-up was 8.9years (0.01–14). At last visit, 96% of patients were in NYHA 1-2 with mostly (71%) normal univentricular function.

Mid-term outcome after extracardiac TCPC is excellent with a good functional result and appears not to be influenced by ventricular morphology. Further studies are needed to evaluate the possible impact of ventricular morphology on late outcome after TCPC.