Case
We present a case of a female patient referred at 10 days of age with 22q11.2 deletion syndrome, TAC type A2 of the van Praagh classification with a regurgitant, quadricuspid TV and VSD (figure 1). The patient had signs of heart failure and respiratory insufficiency and had to be intubated and transferred to the pediatric intensive care unit (PICU) preoperatively. After hemodynamic stabilization surgery was performed.
Median sternotomy is performed, and anatomy is confirmed. The pulmonary arteries are mobilized as much as possible. To achieve cardiopulmonary bypass (CPB), the arterial cannula is inserted in the aortic arch, venous cannulas in the superior and inferior venae cavae. After starting CPB and induction of ventricular fibrillation, the right atrium is opened and cold cardioplegic solution is infused into the coronary sinus under pressure control (Buckberg´s solution, 30 ml/kg/ body weight induction, pressure <30 mmHg). This is followed by the transverse opening of the trunk vessel with a distance to the branches of the coronary vessels. Inspection of the truncus valve, in our case it was severely incompetent in the preoperative transesophageal echocardiography (TEE), showed four cusps and asymmetrical sinuses. Three sinuses and the associated valve leaflets are roughly the same size, the fourth sinus and corresponding valve leaflet are much smaller and, most importantly, have no relation to a coronary ostium. The hypoplastic valve leaflet is resected together with the sinus of the trunk vessel up to the commissures of the adjacent valve cusps. (Figure 2)
These adjacent commissures are then sutured longitudinally starting from the valvular ring with a continuous 7-0 polypropylene suture.
Care must be taken that through the resection of the cusp and the corresponding sinus no valvular stenosis is caused. In our case, the ring diameter was -1 Z score after reconstruction. The further course of the operation is carried out as standard with resection of the pulmonary vessels from the trunk and connection to the RVOT by means of a REV maneuver.
Weaning of CPB and chest closure is performed in a usual manner.
Following surgery, the patient showed a good result and satisfactory recovery. The patient was discharged from the hospital at the 3rd postoperative week. Postoperative echocardiography revealed a good result without truncal valve stenosis or regurgitation. (Figure 3)