[Direct aortic reimplantation for BWG syndrome including mitral valve repair].

Direct aortic reimplantation is the ideal treatment for the BWG syndrome, but is often impossible to obtain a sufficient length of the coronary artery without undue tension on the vessel. We experienced a 6-year-old boy whose left coronary artery was originated from pulmonary trunk widely distant from aorta. Direct reimplantation was successfully performed taking care of excising the ostium of the anomalous vessel as a largest flange and mobilizing the coronary artery from the adjacent tissue sufficiently. Mitral valve regurgitation is also a poor prognosis factor of BWG syndrome. We performed direct reimplantation and mitral valve repair (anterior commissuroplasty) simultaneously to a 3-year-old boy of BWG syndrome with severe mitral valve regurgitation. Postoperative data showed improvement of cardiac performance. Possibility of recovering the papillary muscle function by valve repair without replacement was suggested.