Continuous Murmur Following Mustard Operation for Transposition of the Great Arteries

This patient was seen for the first time at Children's Hospital of Pittsburgh at 34 days of age with cyanosis and grunting respirations of two weeks duration. Cardiac catheterization and angiography confirmed the diagnosis of transposition of the great arteries (TGA) with an intact ventricular septum. Following balloon atrial septostomy, systemic arterial saturation rose to 80% from 34%. Repeat cardiac catheterization at five months and again at two years of age demonstrated normal pulmonary artery pressure and pulmonary vascular resistance. At 3 3/12 years of age she underwent a Mustard operation using cardiopulmonary bypass with moderate hypothermia. Pericardium was used for the baffle. After this baffle had been placed, a small central portion of redundant baffle was reefed with a running horizontal mattress suture. The immediate postoperative course was uncomplicated and the patient was discharged two weeks after operation in excellent condition. Ten months after surgery exertional dyspnea, fatigability, and mild cyanosis appeared. Thirteen one patient showed that the murmur originated at the site of obstruction in the surgically-constructed pulmonary venous atrium. Selective cineangiograms demonstrated the baffle to be the cause of the obstruction. A continuous murmur in a patient following Mustard operation may suggest significant pulmonary venous obstruction.