A four-year-old male patient on the post-natal
first day echocardiogram, unbalanced atrioventricular
channel, single ventricle, pulmonary atresia, and
double outlet right ventricle (DORV) were detected.
The patient who was assessed at the council was
not found to be eligible for biventricular repair. Due
to the cardiac pathologies, the patient underwent
left-sided modified Blalock-Taussig (MBT) shunt
operation, when he was two days old. Pulmonary
artery development was found to be deficient during
the postoperative catheterization. Therefore, right MBT shunting was applied to the patient at the age of
three. In further years, due to the limited pulmonary
arterial development and occluded right MBT shunt
which were detected during clinical follow-up via
transthoracic echocardiography (TTE) and cardiac
catheterization, and a new right MBT shunt procedure
was performed for the third time at the age of seven.
Routine physical examination at the time of hospital
admission revealed a 2/6 systolic murmur at the
pulmonary valve area. Central cyanosis was also
present on physical examination. Thoracic computed
tomography (CT) showed malposition of the great
arteries, atrial septal defect (ASD), ventricular septal
defect (VSD), DORV, pulmonary stenosis, and left
persistent VCS (LPVCS). The preoperative TTE
results also supported the CT findings which showed a
difficult access to the left pulmonary artery (Figure
1).
Figure 1: A computed tomography image of left pulmonary
artery.
In the final cardiac catheterization, the
McGoon index was measured as 1.8 and both left
and right pulmonary arteries were measured as
12 mm. Pulmonary artery pressures were found to be
15/9/12 mmHg. Therefore, the heart team decided to perform a Glenn shunt operation. A written informed
consent was obtained from each parent.
The patient was taken to the Glenn shunt
operation. Median sternotomy was performed under
endotracheal general anesthesia. Adhesions were removed with blunt dissection. Bilateral VCS were
reached and circled. The right MBT shunt was
reached and turned. The left pulmonary artery was
in a compelling position to be operated beneath the
left atrium. First, the right VCS was amputated
from where it merged with the right atrium. The
amputated stump at the right atrium was, then,
closed with primary sutures. The right VCS was
anastomosed in an end-to-side fashion to the right
pulmonary artery. The LPSVC diameter was
measured as 12 mm in width with 22 mmHg pressure intraoperatively. Therefore, LPSVC was ligated in
the distal line and planned to be anastomosed with
the right VCS. Nevertheless, due to the lack of
enough length, the anastomosis was able to perform
via an 8-mm polytetrafluoroethylene (PTFE) ring
graft. We ligated the distal part of the LPVCS and
right MBT shunt. Following the bleeding control, a
drain was placed in both thoraces. The operation was
terminated by closing the layers appropriately to the
anatomy (Figure 2).
Figure 2: An introperative view of surgical field after
procedure.
The patient was intubated and taken to the
intensive care unit. He was hemodynamically stable
and did not need any inotropic support. We started
antiaggregant and low-molecular-weight heparin
(LMWH) treatment to the patient. He was extubated
five hours after surgery. At the end of the first day,
he was transferred to the ward and discharged on
the postoperative eighth day. During discharge, we
discontinued the LMWH treatment and continued
with antiaggregant therapy. One month after the
operation, control TTE showed that and the Glenn
shunt was still functioning. Control CT angiography
showed the 8-mm PTFE graft and patent Glenn shunt
anastomosis (Figure 3, 4).
Figure 3: A postoperative three-dimensional computed
tomography image of the PTFE graft between right and left
superior caval veins.
Figure 4: A postoperative three-dimensional computed
tomography image of the PTFE graft between right and left
superior caval veins.
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