Objective: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology.
Methods: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n=44, 42%) or right ventricular morphology (n=59, 58%). The TCPC was performed using an extracardiac conduit (n=84, 82%) or a lateral atrial tunnel (n=19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years).
Results: Early mortality was 1.9% (n=2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/-1 SEM) was 95.6+/-2.5%. Forty-two patients (41%) had prolonged pleural drainage (> or =14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/-1 SEM) were 92.2+/-5.0 and 73.4+/-6.0%, respectively. The Fontan procedure was associated with an improved functional class (P<0.005) and all current survivors (n=99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P<0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P<0.05), increased postoperative pulmonary artery pressures (P<0.005) and an unfenestrated Fontan procedure (P<0.01).
Conclusions: In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival.