Fontan completion in patients with atrial isomerism and separate hepatic venous drainage

Fontan completion in patients with atrial isomerism, in which the inferior vena cava (IVC) and the hepatic vein (HV) drain separately, is technically challenging. Herein, we review our surgical approach to these patients.

The medical records of 50 consecutive patients with atrial isomerism who underwent Fontan completion between 1998 and 2008 were reviewed retrospectively.

Separate HV drainage was present in 17 patients. Patients with interrupted IVC were excluded. Patient characteristics were as follows: median age, 26 months (range 15-149); median weight, 9.6 kg (range 8.1-47.2); right atrial isomerism, 16 patients; and left atrial isomerism, one. The IVC and the separate HV at the level of diaphragm were contralateral in 16 patients, and ipsilateral in one. The surgical procedures for directing blood flow from the IVC and the separate HV to the pulmonary arteries were as follows: en bloc resection of the IVC and the HV and anastomosing these veins to an extracardiac conduit in 10 patients; connecting the IVC to the HV in a side-to-side fashion before anastomosing them to an extracardiac conduit in one; and lateral tunnel in another. When the IVC and the HV were widely separated by the vertebrae, we chose an intra-extracardiac conduit (intra-atrial septation) in four patients and an extracardiac conduit for the IVC and the right HV and lateral tunnel for the separate left HV in one. There was no mortality. Five re-operations were performed (pacemaker in two patients; one each of fenestration, release of outflow obstruction and ligation of collateral arteries). Sixteen patients underwent follow-up catheterisation, which revealed central venous pressure of 12.0 + or - 2.0 mmHg and arterial oxygen saturation of 92% + or - 6%.

The mid-term results of the Fontan completion in patients with atrial isomerism and separate HV drainage were excellent. The distance between the IVC and the separate HV and the position of the vertebrae should be considered when choosing a surgical technique.