A female neonate with a postnatal diagnosis of complete transposition of the great arteries (TGA) and Rastelli A-type complete atrioventricular septal defect (AVSD) was referred to our institution. Cardiac catheterization and echocardiography revealed an anteroposterior relationship between the great arteries (right anterior aorta and left posterior pulmonary artery) (Fig. 1a). An echocardiography-based preoperative diagnosis of the coronary artery pattern determined that the neonate had a Shaher type 2A classification, with the left anterior descending branch (LAD) originating from sinus 1 and the common trunk of the left circumflex branch (LCx) and right coronary artery (RCA) originating from sinus 2. Both ventricles had balanced volumes (Fig. 1b) and good function.

Preoperative echocardiography imaging. a The aorta was positioned right anterior, and the pulmonary artery was positioned left posterior; Ao aorta, PA pulmonary artery. b Four-chamber view showing balanced ventricles; LV left ventricle, RV right ventricle

Because of the infant’s low body weight, a single-stage complete repair was deemed too difficult; therefore, a staged approach was selected. As initial palliative measures, an arterial switch operation (ASO) and main pulmonary artery (PA) banding were performed via median sternotomy when the neonate was 22 days old and weighed 2.5 kg. Accordingly, a cardiopulmonary bypass was established using aortic and bicaval cannulations. A search for coronary arterial origins showed that the orifices of the RCA and LCx originated from sinus 2, but the location of the LAD orifice as visualized from outside the aorta was not apparent, suggesting that the LAD ran an intramural course. After initiating cardioplegic arrest, the ascending aorta was transected, and the orifice location of each coronary artery was accurately identified (Fig. 2). Both coronary orifices originated from sinus 2 and were close together; therefore, the correct diagnosis was atypical Shaher type 5. The LAD orifice was incised and enlarged to unroof the intramural segment. The posterior commissure was detached from the aortic wall. Both coronary orifices were resected as one large cuff, as these could barely be divided into two separate cuffs and were then re-implanted into the neo-aortic root according to the Mee procedure (Fig. 3) [1]. PA reconstruction was performed using the LeCompte maneuver [2]. Withdrawal from cardiopulmonary bypass was uneventful. After decannulation, PA banding was performed on the arterial circumference (body weight + 19.5 mm). Postoperative hemodynamics were stable, but the PA banding was removed following delayed sternal closure due to mild peripheral PA stenosis.

a Intra-operative findings of coronary arterial origins during the first palliative procedure. The black arrow shows the orifice of the left anterior descending branch, and the blue arrow shows the orifices of the left circumflex branch and right coronary artery. b Schematic representation of the relationship between the great arteries and coronary arteries. The red arrow represents the intramural region of the left anterior descending branch. Ao aorta, PA pulmonary artery, RCA right coronary artery, LAD left anterior descending branch, LCx left circumflex branch

Schematic representation of the coronary artery re-implantation during the arterial switch operation. a The schema displays the origins of the coronary artery, and the red dotted line represents the incision line of coronary cuffs. b Each coronary cuff is divided, and the orifice of the left anterior descending branch is incised and enlarged to unroof the intramural segment. RCA right coronary artery, LAD left anterior descending branch, LCx left circumflex branch

Two months after the ASO, cardiac catheterization indicated that the left ventricle (LV)/right ventricle (RV) end-diastolic volume was 118%/90% of normal. The LV/RV ejection fractions were 61% and 68%, respectively. In addition, the (mean) PA pressure was 29/7 mmHg (12 mmHg).

The patient’s body weight increased sufficiently; subsequently, AVSD repair was performed as an intracardiac procedure once the patient turned 1.3 years old and weighed 8.8 kg. Concomitantly, PA augmentation using an autologous pericardial patch was performed to address the PA bifurcation stenosis (Fig. 4a). As the ventricular septal defect was small (approximately 3 mm), the AVSD was repaired using a modified one-patch procedure [3]. The primary atrial septal defect was closed using an expanded polytetrafluoroethylene patch (Fig. 4b).

Intra-operative findings during the second procedure to completely repair the atrioventricular septal defect. a Pulmonary artery augmentation was performed with an autologous pericardium patch (black arrow). b The atrioventricular septal defect was repaired using the modified one-patch method. The sutures on the edge of the ventricular septal defect were placed through the division line of the common atrioventricular valve to the expanded polytetrafluoroethylene patch (blue arrow) used for the atrial septal defect

The patient’s postoperative course was favorable; she was extubated on postoperative day 1 and discharged on postoperative day 17. Echocardiography performed on postoperative day 14 demonstrated an ejection fraction of 79%, and LV outflow tract obstruction was not observed. Right/left atrioventricular valve regurgitation was mild-to-moderate/none, respectively. The maximum velocity in the right/left PA was measured at 2.6 m/s/1.8 m/s, respectively. The infant is currently a healthy outpatient with no symptoms of heart failure.