Left Main Coronary Artery Atresia Repair in an Infant

In this case, a four-month-old infant presented with cardiogenic shock. He had previously undergone repair of coarctation of the aorta through left thoracotomy at one month old. An aortogram revealed mild anatomical narrowing at the repair site with no gradient across the anastomoses. Aortic root injection revealed that the left anterior descending artery and circumflex artery were filling retrogradely with no communication with the aorta or pulmonary artery. Selective injection of the right coronary artery showed retrograde filling of the left anterior descending artery and the left circumflex artery with faint filling of the left main coronary artery.

The procedure was performed through a median sternotomy. To begin, cardiopulmonary bypass was initiated with right atrial cannulation. A vent was placed into the left ventricle through the right superior pulmonary vein. The superior vena cava was cannulated and the right atrial cannula was transferred to the inferior vena cava. The main pulmonary artery (MPA) was then opened transversely just proximal to the bifurcation. The sinuses of the MPA were carefully examined to exclude anomalous origin of the left main coronary artery (MCA) from the pulmonary artery (PA). The MPA was then transected completely. 

Next, the epicardial course of the left MCA and branches were carefully analyzed. The epicardium over the left main artery was carefully released. The right atrium was opened and a retrograde cardioplegia catheter was placed in the coronary sinus. The ascending aorta was then cross clamped and uniform myocardial protection was achieved by administering del Nido cardioplegia in a retrograde fashion. 

The ascending aorta was then transected. A dimple was seen at the usual site of the left main coronary ostia and surgeons found that a probe could not be passed into the left main coronary artery. A cut back was then made into the aorta across this dimple. The left MCA was opened longitudinally with a Beaver blade. Once the lumen was entered, fine scissors were used to lay the left MCA open from its blind end to the bifurcation. The blind pouch was disconnected from the aortic side, resulting in a spatulated end. A series of interrupted sutures were then used to approximate this spatulated end to the nadir of the aortotomy cut-back incision. These sutures were tied down to form the floor of the neocoronary orifice. 

Next, a long narrow patch of autologous pericardium was treated with glutaraldehyde and used to reconstruct the roof of the left MCA. Suturing began at the apex of the coronary arteriotomy. A 1 mm probe was passed through the coronary artery while taking the apical sutures on the coronary artery to prevent catching the back wall. Suturing was then carried on either side of the coronary artery. The patch was then sutured to either side of the aortotomy cut-back, thereby reconstructing the aortic sinus. 

Finally, the aortic ends were brought together in an end-to-end fashion and the aortic cross clamp was removed. The suture line was examined for bleeding spots. The MPA was brought together in a direct end-to-end fashion. The left PA was then mobilized to create a tension-free anastomoses and prevent the MPA from falling back on the reconstructed left MCA.

References

1. Gebauer R, Cerny S, Vojtovic P, Tax P. Congenital atresia of the left coronary artery--myocardial revascularization in two children. Interact Cardiovasc Thorac Surg. 2008 Dec;7(6):1174-5. doi: 10.1510/icvts.2008.184317. Epub 2008 Aug 26. PMID: 18728035.

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