a two - day - old male with whole body cyanosis and tachypnea born at 38 + 2 weeks of gestation with a birth weight of 4,000 g was referred to samsung medical center .
his percutaneous oxygen saturation was 60% to 70% , and there was no response to oxygen supply .
a grade 3/6 systolic murmur was heard at the left sternal border , and the vital signs were a blood pressure of 70/40 , heart rate of 165 beats per minutes , respiratory rate of 42 breaths per minute , and temperature of 37.2c .
echocardiographic findings demonstrated a complete transposition of the great arteries ( tga ) , large peri - membranous ventricular septal defect ( vsd ) , mild pulmonary stenosis ( ps ) with a peak pressure gradient of 23 to 32 mmhg , and a small patent ductus arteriosus ( pda ) .
additionally , there was subpulmonic deviation of the outlet septum with a small asymmetric pulmonary valve ( pv ) with fusion .
the pv size was 5.86.1 mm ( z - score < 2.5 ) ( fig .
after the procedure , the respiration rate stabilized and the percutaneous oxygen saturation was 80% under ambient conditions .
the patient was relatively well until five months of age when he started to become increasingly short of breath .
eventually , he was admitted for operation , with a body weight of 7.4 kg , and underwent pulmonary root translocation with the lecompte maneuver .
after median sternotomy , an inverted y - shaped pericardiostomy was performed . because in situ material was used for the right ventricular outflow tract ( rvot ) reconstruction , the pericardium was not harvested .
after pda division , antegrade cold blood cardioplegia was infused through the aortic root cannulation .
then , the pulmonary artery and its branches were extensively dissected , from the distal attachments into both pulmonary hila , to be mobilized anterior to right ventriculotomy .
the coronary artery pattern was [ 1 ad ; 2 r , cx ] . because of the prominent left anterior descending ( lad ) coronary artery , we planned a lecompte maneuver . without this maneuver ,
after transection of the proximal ascending aorta , the pulmonary artery root was excised below the pv annulus from the left ventricle ( lv ) ( figs .
2a , 3a ) . the main pulmonary artery was moved anteriorly with the lecompte maneuver .
3b ) , the vsd position was confirmed via the right atrium and right ventriculotomy .
a portion of the right ventricular muscle was resected at the conal septum to relieve the lv exit to the aorta .
then , the defect in the pulmonary root was closed with a bovine pericardial patch and 6.0 polypropylene running suture . after an end - to - end anastomosis of the transected aorta
, the vsd was closed with a bovine pericardial patch and 6.0 polypropylene running suture to create the lv - to - aorta tunnel ( fig .
. formation of lv tunneling with this patch during the lv tunneling , we tried to make the patch straightened for the preservation of rv volume .
the pv was a functional bicuspid valve with fusion , and the pv annulus size which was measured intraoperatively using hegar dilator was 9 mm . appropriate pv annulus size according to the patient s body surface area ( z - score ) was 10.5 mm , so we performed commissurotomy .
the rvot was reconstructed with an excised pulmonary artery root and an in situ autologous pericardial patch that had growth potential on the anterior side ( figs .
after the operation , the patient was extubated on the 3rd post - operative day and discharged with no early post - operative complications on the 8th postoperative day .
echocardiographic findings 7 days after the operation showed minimal ps ( mean pg=13 mmhg ) with a pv size of 6.8 mm ( z - score < 2.5 ) , and those 6 months after the operation showed mild ps ( mean pg=22 mmhg ) with a mature pv size of 15.8 mm ( z - score=+1.70 ) ( fig .
residual arterial septal defect , vsd , or insufficiency of the left ventricular outflow tract were not exhibited . at this time
the patient is doing well 9 months postoperatively and has a new york heart association grade of class i.
the rastelli procedure was first performed in 1968   and soon became a standard surgical technique for patients with tga associated with a vsd and ps .
in addition to excellent relief of ps , the rastelli procedure allows for anatomic correction and achieves good anatomic and physiologic results in the short term .
however , late results of this procedure have critical limitations : low long - term survival and an inevitable reoperation on the obstructed conduit .
these problems come from the inability of the conduit to grow and from lvot stenosis [ 57 ] . to overcome the limitations of this procedure , pulmonary root translocation ( prt ) was introduced by da silva et al . .
with the prt technique , the dissected pulmonary root is implanted in the right ventricle outflow tract to allow for growth of the pulmonary artery .
additionally , a more natural lvot is constructed by avoiding making an incision or mobilizing the aorta .
long - term follow - up data demonstrated high long - term survival and adequate pulmonary root growth .
inspired by da silva , we performed a prt on a five - month - old patient with tga , vsd , and ps . during the operation ,
great care was taken to avoid injury to the pv and the mitral valve when removing the pulmonary root . unlike da silva et al . , we employed the lecompte maneuver .
the translocated pulmonary trunk was close to the sternum , which resulted in an increased risk of bleeding during re - intervention .
additionally , we used an in situ autologous pericardium patch during the rvot reconstruction because of the expected growth potential .
however , a major drawback is the possible formation of aneurysmal dilatation of the pericardial hood . in this case ,
questions regarding adequate pv size for pulmonary root translocation have arisen . however , larger numbers of patients and longer follow - up data will be needed to address this point .
pulmonary root translocation with the lecompte maneuver can be a candidate procedure with the aim of preserving the growth potential of the native pv .
 a five - month - old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the lecompte maneuver . 
 this operation has the advantages of maintaining pulmonary valve function , preserving the capacity for growth , and avoiding problems inherent to the right ventricular to pulmonary artery conduit . 
 this patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the lecompte maneuver . 
CASE REPORT
DISCUSSION