Severe superior vena cava syndrome and hydrocephalus after the Mustard procedure: findings and a new surgical approach.

Some degree of obstruction of the superior vena cava is common after the Mustard operation for transposition of the great arteries. This can cause superior vena cava syndrome, which in extreme cases may lead to hydrocephalus in young children. While revision of the intra-atrial baffle can be performed, this procedure is associated with significant morbidity and mortality and may not resolve the problem. We describe an alternative operative approach in a group of patients who had superior vena cava syndrome after the Mustard procedure: anastomosis of the innominate vein to the left atrial appendage. This procedure is not complicated to perform and is safe; symptoms were relieved in all patients.