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Aortico-Left Ventricular Tunnel
Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery
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Aortico-Left Ventricular Tunnel
Definition A short abnormal pathway, beginning in an aneurysmal dilation of the right sinus of Valsalva (rarely, the left), just to the left of orifice of right coronary artery, and passing through upper end of the ventricular septum to open into the left ventricular cavity. This is a rare congenital cardiac anomaly first described by Levy and Lillehei in 1963.
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Suggested Etiology 1. An example of coronary artery fistula
2. Persistence of embryonic rests of 5th arch 3. Intrauterine rupture of sinus Valsalva 4. Early aortic dissection as in Marfan
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Morphology of Aortico-Left Ventricular Tunnel
Mostly, the aortic orifice is anterior and just downstream from commissural level of AV and separated from right sinus of Valsalva by a prominent transverse supravalvar ridge. The tunnel passes directly downward, beside AV and through the junction between aorta and ventricular septum. The tunnel displace the outlet portion of septum into the RV and produce subpulmonary stenosis. A VSD may coexist, and rarely the tunnel may communicate with the infundibulum of RV rather then LV.
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Classification (Hovaguimian)
Type 1 : slit like opening with no valve distortion (24%) Type 2 : large extracardiac aneurysm (44%) Type 3 : intracardiac aneurysm with or without RVOTO (24%) Type 4 : combination of 2 & 3
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Aortico - Left Ventricle Tunnel
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Clinical Features & Natural History
Incidence ; 0.1% of all CHD Usually have severe AR, & symptoms & signs of severe AR. 3. The age and severity of symptoms are related to the size of the tunnel and the magnitude of the AR. 4. Some patients present as neonate and others with a smaller tunnel may present beyond 2nd decade of life. 5. When symptoms are present, the natural history thereafter is that of severe AR.
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Operation of Aortico-Left Ventricular Tunnel
Indication The diagnosis is an indication for operation and the operation should be performed as early as possible. Technique If possible, both ends of the tunnel are closed, aortic end is closed by placing a pericardial patch, and ventricular end is closed by suture or patch. Particular care is taken to avoid left ventricular distention. Results Hospital death : 5-20% Most patients have at least mild AR. The reason for postoperative AR is poorly understood.
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