Extracardiac Fontan Operation for Right Isomerism, Left-Sided IVC, and Apicocaval Juxtaposition

The patient is a five-year-old girl weighing 21 kg with right isomerism, right-sided superior vena cava (SVC), and left-sided inferior vena cava (IVC). The patient’s pulmonary venous confluence drained to the left atrium. She had a dominant right ventricle and double outlet right ventricle. A right-sided bilateral cavopulmonary connection had been performed four years prior. Surgeons planned for a Fontan completion.

Preoperative cardiac catheterization showed good pulmonary vasculature, patent right side cavopulmonary connection, and left-sided IVC with apicocaval juxtaposition. For the ipsilateral method, the IVC conduit would be compressed by the spine, pulmonary venous confluence, and ventricle. For the contralateral method, the IVC conduit would not be compressed if there was adequate pericardial space between the pericardium and pulmonary venous confluence.

First, cardiopulmonary bypass was established with ascending aorta, SVC, and left femoral vein cannulation. The left-sided IVC was identified and dissected out. The right pulmonary vein and pulmonary venous confluence was then dissected. The Gortex membrane between aorta and pulmonary artery, which was put in during the stage II operation, was dissected out. The Gortex membrane was put in to facilitate dissection between aorta and PA during the Fontan operation.

After complete adhesiolysis, there was adequate pericardial space to accommodate the IVC conduit. On testing, the IVC conduit was not compressed by the ventricle or the spine. IVC conduit configuration was straight with gentle curvature and no acute kinking when placed with the contralateral method. The left-sided IVC was mobilized as low as possible to reduce angulation between the conduit and left-sided IVC after anastomosis.

Next, a pulmonary arteriotomy was performed over the inferior aspect of the pulmonary artery. Superior anastomosis between the pulmonary artery and conduit was performed with a beating heart. The left-sided inferior vena cava was transected and the conduit was trimmed. Inferior anastomosis between the left-sided IVC and conduit was performed with an arrested heart. Next, 4 mm fenestration was made on the conduit. The cardiac end of the IVC stump was sutured around the fenestration. The heart resumed beating in sinus rhythm after the cross clamp was removed.

The patient had an uneventful recovery. Upon her six-month follow up, the patient was well with good saturation. An echocardiogram showed good ventricular function and a patent IVC conduit.