Anomalous origin of the left coronary artery from the pulmonary artery is a rare malformation in which the left coronary artery originates from the pulmonary artery. The consequences are variable although, in most cases, this anomaly leads to severe coronary hypoperfusion and left ventricular dysfunction when pulmonary vascular resistances fall in the postnatal period. Surgical correction is indicated as soon as the diagnosis is established. In nearly all cases, the anomalous artery can be excised from its pulmonary origin, mobilized and reimplanted directly into the ascending aorta. In rare circumstances, technical modifications must be used to restore a normal dual coronary perfusion. The operative risk is related mainly to the severity of preoperative left ventricular dysfunction. The current mortality rate is low, but postoperative left ventricular assist device implantation may be necessary in the most severe cases. After successful revascularization, the late results are satisfactory; left ventricular function always recovers; mitral regurgitation, if present, decreases, although reoperation may be necessary for residual ischemic mitral insufficiency.
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