Off-Pump Right Bidirectional Glenn Combined With a Left Kawashima for a Functional Single Ventricle

This is a three-year-old girl with cyanosis and clubbing. She was born with a double outlet right ventricle and a complete atrioventricular septal defect with a straddling left atrioventricular valve. She underwent an initial main pulmonary artery band but was lost to follow-up.

Other associated anomalies include situs ambiguous, left isomerism with polysplenia, and interrupted inferior vena cava with hemi-azygous continuation to a left sided superior vena cava.
In this case, the decision was made to proceed with the current procedure due to the patient’s cyanosis.

The procedure was performed through a repeat median sternotomy without cardiopulmonary bypass. After initial mediastinal dissection, Heparin was administered systemically. The right superior vena cava was divided between two vascular clamps and its cardiac end was oversewn in a two-layer fashion. A side-biting clamp was then applied on the right pulmonary artery and an arteriotomy was made on its posterior-superior border. An end-to-side anastomosis was constructed between the cephalic end of the right superior vena cava and the right pulmonary artery using running 6-0 polypropylene suture along its back wall and multiple interrupted 6-0 polypropylene sutures along its anterior wall to avoid a purse-string effect. All clamps were then removed and hemostasis was achieved on that side. The azygous vein was doubly ligated and divided.

Surgeons then directed their attention to the left side. The left superior vena cava was divided between two vascular clamps and the left cavopulmonary anastomosis was constructed in a similar fashion to the right side.

It was then decided to leave antegrade pulmonary blood flow but further band the main pulmonary artery. This was done using a strip of Gore-Tex graft, and the pressure was measured in both right and left superior venae cavae as acceptable.

The patient's hemodynamics and oxygen saturation remained stable during the procedure and the chest was closed in the standard fashion. The patient tolerated the procedure well and was extubated in the operating room and transferred to the ICU in a hemodynamically stable fashion.

References

1. Nguyen TT, Nguyen DH, Nguyen TC, Luong LH. Bidirectional Glenn operation without cardiopulmonary bypass: Single center experience and results. Asian Cardiovasc Thorac Ann. 2024 Jan;32(1):5-10
2. Liu J, Lu Y, Chen H, Shi Z, Su Z, Ding W. Bidirectional Glenn procedure without cardiopulmonary bypass. Ann Thorac Surg. 2004 Apr;77(4):1349-52
3. Estafanos M, Mashadi A, Said SM. Off-Pump Kawashima Procedure in a 10-Month-Old Infant with Heterotaxy. CTSNet. Thursday, August 17, 2023
4. Said SM, Mohamed A, Shakur Z, Marey G. Extracardiac Fontan completion after the Kawashima procedure with a custom-made bifurcated graft. Multimed Man Cardiothorac Surg. 2022 Oct 11;2022
5. Said S, Marey G. Off-pump extracardiac Fontan completion: Surgical technique and pitfalls. Multimed Man Cardiothorac Surg. 2021 Jan 28;2021

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