Aorto-Left Ventricular Tunnel Neonatal Repair

Introduction

Levy et al [1] first described the aorto-left ventricular tunnel in 1963. This rare congenital malformation is an abnormal channel that connects the ascending aorta with the left ventricle, bypassing the aortic valve. In view of its rarity, surgical series are small. Most of the patients develop symptoms of heart failure during the first year of life, but there are cases of asymptomatic adults [2-3]. Onset of heart failure depends on the cross-sectional area of the tunnel and the amount of aortic regurgitation. We describe the surgical approach in a neonatal patient.

Case Report

The patient was a two-day-old, three-kilogram male, with prenatal diagnosis of aorto-left ventricular tunnel. From birth he presented a situation of heart failure requiring CPAP and inotropes. The echocardiogram showed an aorto-left ventricular tunnel in the right Valsalva sinus of 5mm and an aortic annulus of 8mm. The aortic valve was dysplastic and thickened. The left ventricular function was normal.

Operation Technique and Result

We performed a median sternotomy. There was a big aneurysm of the right Valsalva sinus. Bicaval cannulation was established. A vent was placed in the right superior pulmonary vein. Mild hypothermia at 28° was reached. The aorta was clamped and anterograde cardioplegia with external occlusion of the tunnel was infused. The aorta was incised transversely just above the sinotubular junction. Extra cardioplegia was delivered to the right coronary artery. An enlargement of the aortotomy was made in the aneurysmal sinus. An equine patch was used to close the ventricular opening of the tunnel with running polypropylene suture. The ventricular aneurysm was excluded. The patch was anchored to the aortic annulus. A second equine patch was used to close the aortic opening of the tunnel in a T-shape orientation. Removal of the aneurysmal tissue from the aortic wall was accomplished, and the aortic root was closed with a running polypropylene suture. A second-layer suture was performed to ensure good hemostasis. The aortotomy was closed with a running suture. A right atriotomy was made in order to close the atrial septal defect. An equine patch was used with a running suture. The right atriotomy was closed. The postoperative echocardiogram showed no residual leak of the tunnel closure and there was no significant gradient or regurgitation of the aortic valve.

The chest was left open until the third postoperative day. The patient was extubated and inotropes were withdrawn 9 days and 13 days after surgery, respectively. The ICU stay was 18 days. The postoperative course was uneventful. The patient was discharged home on postoperative day 28. The echocardiogram at 6 months of follow up remained without residual leak and with mild aortic valve regurgitation without significant gradient.

References

1. Levy MJ, Lillehei CW, Anderson RC, Amplatz K, Edwards JE. Aortico-left ventricular tunnel. Circulation. 1963;27:841-853.
2. Kafka H, Chan KL, Leach AJ. Asymptomatic aortico-left ventricular tunnel in adulthood. Am J Cardiol. 1989;63(13):1021-1022. doi:10.1016/0002-9149(89)90168-9
3. Mueller C, Dave H, Prêtre R. Surgical repair of aorto-ventricular tunnel. Multimed Man Cardiothorac Surg. 2012;2012:mms006. doi:10.1093/mmcts/mms006

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