MOYAMOYA SYNDROME-ASSOCIATED WITH CONGENITAL HEART-DISEASE

Objective. To describe the association between moyamoya syndrome and c ongenital heart disease and to discuss its clinical implications. Stud y Design. Retrospective analysis of a case series from two institution s. Results. Five patients with moyamoya syndrome and structural congen ital heart disease were identified. Coarctation of the aorta was prese nt in 3 patients, in association with a ventricular septal defect (1 p atient), aortic and mitral valve stenoses (1 patient), and tetralogy o f Fallot (1 patient). Tetralogy of Fallot and a large paramembranous v entricular septal defect were found in the other 2 patients. Four pati ents underwent surgical repair of their congenital heart disease durin g the first year of life and 1 patient had balloon dilation of aortic coarctation at 5 years of age. In all patients, moyamoya syndrome was diagnosed after surgical intervention for congenital heart disease-at 6 months of age in 1 patient, at 2 years of age in 3 patients, and at 6 years in 1 patient. Strokes were the most common presenting sign (3 patients) followed by seizures (2 patients). By the age of 33 months, 4 of 5 patients had undergone cerebral revascularization surgery to ha lt the clinical progression of moyamoya syndrome. Conclusions. Moyamoy a syndrome should be considered in the differential diagnosis of seizu res and stroke in patients with structural congenital heart disease. P rompt diagnosis and surgical management of the occlusive cerebral angi opathy should lead to improved neurological outcome in these patients.