A 2 kg girl was transferred from another hospital to the ICU of Siyami
Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital
with a preliminary diagnosis of a hypoplastic left ventricle (AA/MA)
and supracardiac TAPVR. The echocardiography showed mitral atresia,
aortic atresia, 1 mm diameter ascending aorta, and mild stenosis in a
vertical vein and pulmonary venous confluence. The patient was intubated
and general condition was poor. A bilateral pulmonary banding was
performed and the PGE infusion was continued to keep the PDA open. In
the follow-up period following the pulmonary banding procedure, sepsis
developed in the ICU. After the medical treatment was completed, it was
decided that the patient should be operated on to address the increased
stenosis in the pulmonary venous return. The chest X-ray showed
pulmonary edema. Considering poor cardiac function, pulmonary edema, and
a 1 mm ascending aorta in diameter, the decision was made to do the
surgery on beating heart. Interventional procedures to TAPVR were not
possible due to the multiple stenoses in the vertical vein and pulmonary
venous confluence.
Since the hybrid procedure is not possible unless the pulmonary
venous return is corrected, it was decided to modify the hybrid
procedure by inserting a graft into the PDA instead of stenting the PDA
(1). The mediastinum was reached through the previous incision. The
innominate artery was cannulated with an 8F cannula. A bicaval
cannulation was performed. Previously banded pulmonary arteries were
dissected and hanged with silastic tapes after initiating a bypass. The
procedure was performed under 32 degrees Celsius normothermia. Pledgeted
traction sutures were placed at the apex of the heart. The heart was
pulled towards the patient's right shoulder. The posterior pericardium
was opened parallel to the left phrenic nerve. Pericardial traction
sutures were placed. A descending aorta dissection was completed and
freed from the esophagus (2). Purse sutures were placed in the
descending aorta and cannulation was completed by using a side clamp.
Since
the patient had aortic atresia, it was decided to correct TAPVR without
placing a cross-clamp. The right atriotomy was done. The location of
the pulmonary venous chamber was evaluated externally. Atrial septum was
opened with a scalpel. The left atrium was very small. The right
atriotomy incision was extended toward the left atrium and biatrial
incision was completed (3-4). The venous confluence was opened with a
scalpel. The venous confluence was anastomosed to the atrium. The atrium
was closed in a double-layer fashion. Branch vessels of the aortic
arch, PDA, and descending aortic dissections were completed.
Cross-clamps were placed on the descending aorta and the proximal aortic
arch. A pulmonary arteriotomy was done transversely below the pulmonary
artery orifices. The 6 mm Hegar dilator was inserted into the PDA. In
the preoperative measurements, PDA was seen as 6 mm in diameter and 1 cm
in length. Therefore, a decision was made to use a 6 mm ringed PTFE
graft. The ringed graft was preferred to prevent kinging. A Hegar
dilator was inserted into the PDA and the graft length to be used was
calculated and marked. After inserting the graft into the PDA, it was
fixed with 7/0 prolene sutures to the pulmonary artery side. The
proximal and distal aortic arch was checked with a right-angle clamp
inserted through the graft. The right and left pulmonary artery orifices
were evaluated and found to be open. The pulmonary arteriotomy was
closed. The cannula in the descending aorta was removed and its sutures
were tied. The bypass was completed without inotropic support. The MUF
was done. It was decided that the previously placed tapes were
sufficient and should not be tightened anymore. The patient was
decannulated and the sternum was temporarily closed with a patch. There
was no pressure difference between the femoral artery and the right
radial artery. No obstruction was detected in the pulmonary venous
return on transthoracic echocardiography. There was no flow acceleration
in the PDA or significant gradient in the aortic arch.
References
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Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, et al. Stenting
of the arterial duct and banding of the pulmonary arteries: basis for
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JM. Supplemental perfusion techniques for aortic arch reconstruction,
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K, Wang H, Wang Z, Zhu H, Fang M, Zhu X, et al. Early- and
intermediate-term results of surgical correction in 122 patients with
total anomalous pulmonary venous connection and biventricular
physiology. J Cardiothorac Surg. 2015;10:172.