Patient profile
A 10-year old boy, diagnosed with right ventricular double outlet (Taussig-Bing type) at 2 months after birth, received arterial switch procedure with ventricular septal defect repair via the median sternotomy. He was admitted to our hospital with a 1-month history of refractory hypertension. His body weight was 40kg. Physical examination revealed an arm-leg systolic pressure differential of 70mmHg. His femoral pulses were faint and pedal pulses were absent. Diastolic murmur along the left sternal border accompanied with wide pulse pressure and peripheral vessel sighs were observed. His chest radiograph was shown inFigure 1 . Computed tomography revealed that the aorta was anterior to the left, and the pulmonary artery was posterior to the right. There was a critical coarctation of the aorta (COA) distal to the left subclavian artery with a luminal diameter of less than 5 mm and extensive collateral thoracic vasculature. Notably, the aortic root was significantly dilated to 65mm measured on 3-dimensional cross-sectional imaging, as present in Figure 2 . Transthoracic echocardiography demonstrated a severe insufficiency of tricuspid aortic valve and a significantly dilated aortic annulus. His trans-coarctation pressure gradient was up to 76mmHg. Written informed consent with the approval of our institutional review
board was obtained from the patient’s family before performing the
procedure.
Extra-anatomic ascending-descending aortic bypass and Bentall procedure was performed with median sternotomy in a single-stage operation. Cardiopulmonary bypass (CPB) was instituted via the distal portal of the ascending aorta and both caval cannulation. Intraoperative transesophageal echocardiography and inspection of aortic root revealed that one leaflet of the tricuspid aortic valve underdeveloped with significant insufficiency, not allowing for valve-sparing root replacement. A 23mm mechanical valve with aortic tube graft was employed to replace the aortic valve, aortic root, and proximal portion of the ascending aorta. Coronary ostia were re-implanted into the neo-aortic graft. Before the root portion of the procedure was performed, a 20-mm woven Dacron graft was anastomosed to the descending thoracic aorta via a posterior pericardial approach. After completion of the Bentall procedure, the descending graft was positioned around the left border of heart and anastomosed to the neo-ascending aortic graft. The patient was weaned from CPB uneventfully. Total aortic cross-clamp time was 184 minutes and CPB time was 329 minutes. Monitoring of radial and femoral arterial line pressures demonstrated no residual arm-leg pressure gradient.
The patient was discharged without any major complications. Post-operative transesophageal echocardiography revealed functional prosthetic valve and preserved left ventricular systolic function. Postoperative chest radiograph and computed tomography demonstrated that the extra-anatomic aortic bypass graft ran along with the left border of the heart and was patency (Figure 3&4 ). During 52-months of follow-up, there was a significant reduction in mean upper-extremity blood pressure after operation without medication.