Modified Yasui Procedure for Interrupted Aortic Arch Type B

This is a case presentation of a 9-day-old female infant born at 38 weeks with a postnatal diagnosis of interrupted aortic arch type B, two large posterior malaligned ventricular septal defects, severe left ventricular outflow tract narrowing, and a large patent foramen ovale. 

Additional noncardiac findings indicated an absence of thymic tissue, correlating with clinical findings suggestive of DiGeorge syndrome. The patient was born to a group B Streptococcus-positive mother with associated fever at 38 weeks and had been receiving prostaglandins since the cardiac diagnosis.  

A 3D rendering of the patient’s heart was created to highlight key anatomical features and assist in the complex surgical planning needed for a successful operation. Clearly visible in the rendering were the patient’s interrupted aortic arch and the large posterior ventricular septal defects. 
 
Preoperative transthoracic echocardiogram (TTE) indicated an interrupted aortic arch type B, a hypoplastic aortic valve (4.2 mm, z-score -3.6), a severely narrowed left ventricular outflow tract measuring 3.6 mm in diameter, and an abhorrent left subclavian artery originating from the rightward aspect of the ductal insertion on the descending aorta. The cardiothoracic surgical team advocated for a Yasui procedure under deep hypothermic circulatory arrest to address the interrupted aortic arch. 
 
Procedure 
 
The pericardium was opened in a stellate fashion to further mobilize the ascending aorta, branch pulmonary arteries, and the ductus arteriosus leading to the descending aorta and left subclavian artery. The patient was dual cannulated through the lateral aspect of the ascending aorta and the proximal portion of ductal arch. The patient was then heparinized, and the right atrial appendage was cannulated with an 18 French right-angle catheter. Cardiopulmonary bypass commenced, and the patient was crash cooled to 15 degrees Celsius. Subsequently, the patient was given 10 cc/kg of antegrade del Nido cardioplegia, and the bypass pump was then ceased when the patient was drained of blood. 
 
Attention was then turned to dividing the ductus arteriosus and fully mobilizing the descending aorta. The pulmonary artery was subsequently divided at its bifurcation, and the branch pulmonary arteries were mobilized for superior retraction. An arteriotomy was made on the left lateral side of the ascending aorta. The descending aorta was then anastomosed to the cut edge of the main pulmonary artery on the left lateral side with 7-0 Prolene in a running fashion.  

The arteriotomy made in the ascending aorta was then anastomosed at its base to the right-sided pulmonary artery. This area was patched with pulmonary homograft from the descending aorta over the pulmonary artery into the ascending aorta. A 5 mm dilator was passed into the newly constructed aortic arch, showing no evidence of obstruction. Attention then shifted to the right ventricular outflow tract.  

A ventriculotomy was performed to visualize the posterior malaligned ventricular septal defect. The lateral wall was excised to enlarge the ventricular septal defect while avoiding conduction tissue. A piece of cardio cell was then utilized to augment flow from the ventricular septal defect to the pulmonary valve. It is important to note at this time that the patient was recannulated, bypass was resumed, and warming began.  

A 9 mm homograft was then brought into the surgical field and trimmed appropriately. The distal end of the homograft was then anastomosed to the branch pulmonary arteries with 6-0 Prolene sutures in a running fashion. The proximal portion of the homograft was anastomosed to the ventriculotomy, and a cardio cell patch was used to augment the neo-right ventricular outflow tract with the conduit. 
 
Once the patient was warmed properly, she was separated from cardiopulmonary bypass. Upon separation, hypotension and hypocalcemia were noted, and the patient received a calcium drip. Once adequate hemostasis was achieved, the patient was given protamine and decannulated. The chest was left open, and the skin was closed with a silastic patch before the patient was transferred to the cardiovascular intensive care unit (CVICU) for postoperative recovery. 
 
Postoperation 
 
Postoperative transthoracic echocardiogram (TTE) confirmed the successful surgical operation of the modified Yasui procedure, along with ventricular septal defect enlargement. Visualization showed the ascending and descending aorta connecting separately to the native pulmonary artery trunk. There was a residual patent foramen ovale, mild tricuspid valve insufficiency, and a moderately hypertrophied and depressed right ventricle. 
 
Cardiopulmonary bypass time was 65 minutes, aortic cross-clamp time was 46 minutes, and the deep hypothermic circulatory arrest time was 45 minutes. The patient was discharged from the CVICU on postoperative day 14. 

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