Off-Pump Kawashima Procedure in a 10-Month-Old Infant with Heterotaxy

A ten-month-old, 9.4 kg infant with heterotaxy syndrome presented with cyanosis. The cardiac diagnosis was a double outlet right ventricle with complete atrioventricular septal defect and multilevel right ventricular outflow tract obstruction. Other multiple anomalies included dextrocardia, situs inversus, interrupted inferior vena cava with azygous continuity to a left superior vena cava (SVC), malposed great arteries, small patent ductus arteriosus, and left aortic arch with common origin of the left common carotid artery and innominate artery. Because of the chordal attachment of the atrioventricular valves, it was felt that the patient would be better served with single ventricle palliation.

The Surgery

Through a median sternotomy, a pericardiotomy was performed. The left SVC was dissected and mobilized, followed by identification of the large azygous vein and dissection and mobilization of the left pulmonary artery.

Next, heparin was administered systemically. A right-angled venous cannula was placed in the left SVC, and a straight venous cannula was placed in the common atrium. Both cannulae were then connected via a Y-connector to allow passive gravity-assisted drainage. Test clamping of the left SVC showed a high pressure in the neck veins, so it was decided to convert this to a pump-assisted active drainage. The Y-connector was removed, and the SVC cannula was connected to a small circuit in which the SVC line was the inflow to the pump and the outflow was connected back to the common atrial cannula. This allowed better drainage and facilitated conducting the procedure without the need for full cardiopulmonary bypass.

The left SVC was then divided between two vascular clamps, and its cardiac end was oversewn in two layers with running 5-0 polypropylene sutures. Proximal and distal control was then obtained on the left pulmonary artery and an arteriotomy was made. An end-to-side anastomosis was performed between the left SVC and the PA using running 5-0 polypropylene sutures at the back wall and multiple interrupted 6-0 polypropylene sutures at the front wall of the anastomosis to avoid any purse-string effect. All clamps were then removed, and the anastomosis was deaired.

The patient’s oxygen saturation improved immediately to high 90 percent. All cannulae were then removed and their sites were secured.

The rest of the procedure and chest closure were performed in the standard fashion. The patient was extubated in the operating room, received no transfusion, and the remaining postoperative course was uneventful. She was discharged on the seventh postoperative day. The patient continued to do well during her follow-up.

Reference(s)

1. 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

2. Said S, Marey G. Off-pump extracardiac Fontan completion: Surgical technique and pitfalls. Multimed Man Cardiothorac Surg. 2021 Jan 28;2021

3. Alsoufi B, Rosenblum J, Travers C, Kanter K, Trusty PM, Yoganathan AP, Slesnick TP. Outcomes of Single Ventricle Patients Undergoing the Kawashima Procedure: Can We Do Better? World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):20-27

4. Lively-Endicott H, Lara DA. Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism. Ochsner J. 2018 Winter;18(4):406-412