Case Report
We present the case of a 2,770 gram baby girl born at 37 weeks and 2
days with a prenatal diagnosis of d-TGA with intact ventricular septum
who was initially brought to the neonatal intensive care unit on
prostaglandin. She was then quickly taken for a cardiac catheterization
for a balloon atrial septostomy. TTE confirmed the prenatal diagnosis of
d-TGA with intact ventricular septum and also showed a large left patent
ductus arteriosus (PDA) with bidirectional shunting. Aortic arch imaging
suspected AORSA because of a lack of bifurcation of the first right
brachiocephalic branch, although its origin was not visualized. Her body
appeared cyanotic and dusky with sparing of the right hemithorax and
arm. Pre-ductal oxygen saturation on the right hand was consistently
above 90% whereas the post-ductal oxygen saturations on all other
extremities were 60 – 70%. Interestingly, when the pre-ductal pulse
oximeter was moved to the right ear, the oxygen saturation dropped to
resemble the post-ductal levels. This prompted for systematic evaluation
for the origin of the right subclavian artery from the pulmonary artery
system, in the setting of transposition physiology. Repeat TTE confirmed
prior findings and clearly demonstrated the right subclavian artery from
the proximal RPA. Additional anatomic details included anomalous origin
of the left coronary artery from the right sinus and an isolated cleft
of the mitral valve that was diagnosed on fetal imaging. On day of life
13, the patient underwent an arterial switch operation (ASO) with
LeCompte maneuver, single coronary button with creation of a neo-aortic
to coronary hood, closure of atrial septal defect, and ligation of the
left-sided PDA. The preoperative echocardiographic diagnosis of AORSA
from the proximal RPA was confirmed (Figure 1). The anomalous vessel was
ligated and divided from the RPA. She had an uncomplicated
post-operative course.