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A five-year-old girl, weighing 15.8 kg, with a functional single ventricle (hypoplastic left heart syndrome secondary to aortic and mitral valvular atresia), was referred for a completion Fontan procedure. Her past surgical history included an initial modified Norwood/Sano procedure in the neonatal period, followed by stage II palliation with a bidirectional cavopulmonary anastomosis at eight months of age. She also had mosaic Turner syndrome.
Preoperative work-up included echocardiography, cardiac catheterization, and computed tomography, all of which confirmed her suitability for the completion Fontan. The procedure was performed via a repeat median sternotomy and without the use of cardiopulmonary bypass. An 18 mm extended polytetrafluoroethylene (ePTFE) graft was used as the extracardiac Fontan conduit. The graft was anastomosed to the left pulmonary artery in an end-to-side fashion.
The inferior vena cava (IVC) was cannulated with a right-angled cannula, and a straight cannula was placed through the common atrium. Both cannulae were connected with a Y-connector to create a passive drainage circuit for the IVC. This helps maintain hemodynamics during the clamping of the IVC and the construction of the IVC to graft anastomosis.
The patient was extubated in the operating room and received no transfusions. The postoperative course was overall uneventful, and she continued to do well during her follow-up.
References
- Said S, Marey G. Off-pump extracardiac Fontan completion: Surgical technique and pitfalls. Multimed Man Cardiothorac Surg. 2021 Jan 28;2021. doi: 10.1510/mmcts.2021.004.
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