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)