A 5-year-old girl weighing 17.4 kg who had single ventricle physiology was referred to our hospital from Kazakhstan for a Fontan operation. She was diagnosed in her country with DORV (TGA type), PS, and situs inversus totalis. At the time of her birth, her pulmonary valve and LV were not adequate to support the systemic circulation because of moderate valvar and subvalvar stenosis and a relatively small LV cavity. Therefore, the doctors in Kazakhstan selected the single ventricle palliation course of treatment for the infant. At 4 months of age, she underwent a left-sided modified Blalock-Taussig shunt, patent ductus arteriosus ligation, and atrial septectomy performed by a local medical team in Kazakhstan. At 2 years of age, shunt takedown, left bidirectional cavopulmonary shunt (BCPS), and main pulmonary artery (PA) division were performed in Kazakhstan by our medical team volunteers who visited Kazakhstan every year to follow up on the patient. Annual echocardiography of the patient showed that the LV size was growing too adequately to persist with the single ventricle palliation course of treatment. We thought that the patient might be able to undergo biventricular repair, but precise examinations, such as computed tomography angiography with 3-dimensional reconstruction, and a multidisciplinary approach were required. We decided to invite her to our hospital in South Korea and formulated the next management plan for her.
Chest radiography showed situs inversus totalis with dextrocardia (Fig. 1). Echocardiography demonstrated DORV (TGA type) with an anterior aorta, a large VSD, mild tricuspid regurgitation, and laminar flow from the superior vena cava (SVC) to the PA. The indexed LV end-systolic and end-diastolic volumes were 5.7 and 11.6 ml/m2, respectively. The mitral valve Z-score was − 1.76, and the mitral valve/tricuspid valve ratio was 0.87. The LV ejection fraction obtained using the Simpson method was 59.2%. A computed tomography angiography scan showed the same anatomical results with echocardiography (Fig. 2). Moreover, an inferior vena cava interruption with azygous continuation and hepatic veins draining directly into the right atrium was observed. Via a multidisciplinary approach, we considered her LV to be suitable for biventricular repair; thus, HTTSO was planned.
The operation was performed under routine cardiopulmonary bypass with the use of a Bretschneider histidine-tryptophan-ketoglutarate crystalloid solution, bicaval cannulation, and mild hypothermia induction. The technique for the HTTSO was similar to that described in our previous report [3]. In brief, the aorta was transected 10 mm above the coronary arteries and Yacoub type A coronary arteries were harvested. When the main PA stump was opened, the pulmonary valve was found to be bicuspid, and the valve leaflets appeared dysplastic, edematous, and thick. After truncal block resection, the VSD was closed using a bovine pericardial patch with interrupted 6–0 polypropylene double pledget supported sutures. The truncal block was half turned horizontally, and the posteriorly translocated aortic valve was anastomosed to the LVOT with 6–0 polypropylene continuous sutures. The coronary buttons were anastomosed to the corresponding aortic wall defects. After the takedown of BCPS, the PA was closed with a bovine pericardial patch. The posterior wall of the proximal PA stump was directly anastomosed to the posterior wall of the PA bifurcation without a Lecompte maneuver. The anterior wall of the neo-RVOT was covered by the bovine pericardial patch with the native valve leaflets. A longitudinal incision was made along the anterior wall of the cephalic end of the SVC to the innominate vein–SVC junction and then anastomosed directly to the right atrial auricle (RAA). This anastomosis was completed with a 7–0 polypropylene running suture, starting at the posterior wall with primary SVC-RAA approximation and anterior patch augmentation with bovine pericardium. Weaning from cardiopulmonary bypass was smoothly performed with the use of a low-dose prophylactic inotropic agent. The duration of the cardiopulmonary bypass and aortic cross clamp were 295 and 185 min, respectively.
The patient was extubated at 1 day after the operation, and the postoperative course was uneventful. The patient was discharged on postoperative day 6 and went back to Kazakhstan. Postoperative echocardiography (Fig. 3) showed a normal LV function, straight flow of the LVOT without stenosis, mild tricuspid regurgitation, no mitral regurgitation, mild pulmonary regurgitation, and mild PS (peak velocity, 3 m/s). She had a normal sinus rhythm and had no stenosis at the SVC-RAA anastomotic site.