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.