Undiagnosed Circumflex Artery Off The Right Pulmonary Artery: The Importance Of Routine Intraoperative Inspection Of Coronary Arteries In Neonatal Heart Surgery
Douglas Overbey, MD MPH, Nicholas Andersen, MD, Joseph Turek, MD PhD.
Duke University, Durham, NC, USA.
Objective(s): A 6 day old female was evaluated for a hypoplastic transverse arch, VSD, and borderline hypoplastic left ventricle with fluctuating function. Norwood was planned due to borderline size and function of the LV.
Methods: During preliminary dissection, a small vessel was noted to be coursing inferiorly from the right PA toward the left atrium (Figure 1). Further anatomic and echocardiographic inspection showed two native coronary orifices, but a left main branching only to a LAD. The inferior vessel from the RPA was test occluded which correlated with echocardiographic flow cessation and consistent EKG changes, confirming a left circumflex ALCAPA. Since the ALCAPA likely contributed to the neonatal waxing and waning of LV function, we opted for a biventricular repair.
Results: Cardioplegia was administered in two phases, first through the native aorta followed by through the PA after distal branch and PDA occlusion. We then created a button from the RPA to include the ALCAPA, which due to size included the entire circumference of the RPA. The LCx button was mobilized and reimplanted posteriorly into the aorta (Figure 2).
Conclusions: Anomalous coronary arteries can be missed on preoperative echo, and careful intraoperative inspection should be performed to appropriately reimplant. 
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