Despite a wide anatomic diversity, the complete repair of all conotruncal anomalies includes two surgical steps. 1) An intracardiac tunnel is created to connect the left ventricle to one of the arterial orifices (usually the aortic, sometimes the pulmonary), through the conoventricular ventricular septal defect. Any conal septum should be resected to create a short, large, and straight tunnel. Abnormal insertions of the atrioventricular valves (tricuspid and mitral) on the conal septum should be preserved. “Intramural” residual ventricular septal defects must be avoided by anchoring the intracardiac patch directly to the arterial annulus. 2) To connect the right ventricle to the pulmonary artery, either an intracardiac or an extracardiac reconstruction is carried out, according to the distance between the tricuspid valve and the pulmonary orifice. When extracardiac reconstruction is indicated, it is usually performed without prosthetic conduit (with or without French maneuver, eventually using the left atrial appendage). In most patients, complete repair can be performed as a primary operation during infancy.
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