Surgical Management of Single Intramural Coronary Artery in Taussig-Bing Anomaly Detected at Arterial Switch Operation

Case Report

The second child of a 21-year-old mother was born at 39 weeks gestation after an uneventful pregnancy. At birth, the baby weighed 2640 g and measured 48 cm. The sole risk factor during pregnancy was maternal smoking. Nine days postpartum, the newborn was found to be tachydyspneic, tachycardic and showed oxygen saturations of 87 - 91 %. Furthermore, it had cool limbs.

Echocardiography showed situs solitus, atrioventricular concordance, ventriculoarterial discordance, a subpulmonary ventricular septal defect (VSD) with at least 50 % overriding of the pulmonary artery. Both great arteries arose from the right ventricle, confirming the presence of a double outlet right ventricle. Biventricular function was normal. Two coronary arteries were detected, and a normal coronary pattern was assumed. An angiocardiography was performed to rule out a suspected coarctation, as the aortic isthmus was not entirely visible on echocardiography in the presence of a large duct with turbulent flow. The coronary anatomy suggested that both ostia were very near to each other, but the precise coronary anatomy remained unclear in all views at angiography ([Fig. 1]). An angiography with a laid back view angulation, which is probably the best method to detect the coronary anatomy in patients with transposition of the great arteries, was not performed.

Fig. 1 Preoperative angiographic finding, showing both coronary arteries. RCA: right coronary artery, LAD: left anterior descending artery.

The decision was made to correct this congenital heart defect by performing an arterial switch operation with patch closure of the VSD. The chest was opened through a midline sternotomy. The exact coronary artery pattern remained unclear. Cardiopulmonary bypass was instituted in the usual manner and the patient was cooled down to a core temperature of 28 °C. The heart was arrested with cold antegrade crystalloid cardioplegia. After transsection of the aorta, the coronary artery pattern was found to be very unusual. There was a large single ostium arising at the height of the sinotubular junction juxtacommisural between the opposing sinuses 1 and 2. The right coronary artery followed its normal course around the aorta, whereas the left coronary artery (LCA) passed intramurally, taking its course between the aorta and the pulmonary artery, then dividing into the circumflex and left anterior descending artery ([Fig. 2]). Because of the proximity of both coronary arteries and the intramural course of the LCA, it was not possible to separate them. To transfer the coronary arteries, we opted for an aortocoronary flap technique using a patch of glutaraldehyde-preserved autologous pericardium as a hood. After extensive mobilization of the coronaries, a U-shaped incision was made in the pulmonary artery wall facing the coronary artery ostium. Then, the ostium was slightly rotated and anastomosed to the pulmonary artery. The superior part of the coronary ostium was enlarged with a patch of glutaraldehyde-preserved autologous pericardium ([Fig. 3]). The VSD was closed with a Gore-Tex® (W. L. Gore & Associates, Inc., Flagstaff, AZ, USA) patch and the pulmonary artery anastomosed with the ascending aorta to form the neoaorta. After the French maneuver, a pantaloon-shaped patch of glutaraldehyde-preserved autologous pericardium was utilized to complete the right ventricular outflow tract. The patient was weaned off bypass without difficulty and the chest was closed primarily. Intraoperative transesophageal echocardiography showed an unobstructed flow into both coronary arteries ([Fig. 4]). The postoperative course was uneventful; the patient was extubated on the second postoperative day and discharged from the intensive care unit on day 5.

Fig. 2 Illustration showing the origin of the single coronary ostium and its course between the great arteries.

Fig. 3 Intraoperative view of the reconstruction of the coronary transfer. RCA: right coronary artery, LCA: left coronary artery, * pericardial hood.

Fig. 4 Intraoperative transesophageal echocardiography showing the branching of both coronary arteries. RCA: right coronary artery, LCA: left coronary artery.

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