Moyamoya syndrome associated with congenital heart disease
Moyamoya syndrome associated with congenital heart disease
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DOI：
10.1542/peds.101.1.57
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发表时间：
1998-01-01
期刊：
影响因子：
8
通讯作者：
Geva, T
中科院分区：
文献类型：
--
作者：
Lutterman, J;Scott, M;Geva, T
Objective.描述烟雾综合征与先天性心脏病之间的关系，并讨论其临床意义。对两个机构的病例系列进行回顾性分析。5例烟雾综合征和结构性先天性心脏病被确定。3例患者存在主动脉缩窄，与室间隔缺损（1例患者）、主动脉瓣和二尖瓣狭窄（1例患者）和法洛四联症（1例患者）相关。另2例为法洛四联症和膜旁室间隔缺损。4例患者在出生后1年内接受了先天性心脏病手术修复，1例患者在5岁时接受了主动脉缩窄球囊扩张术。在所有的患者中，烟雾综合征是在先天性心脏病手术干预后诊断的，1例患者在6个月大时，3例在2岁时，1例在6岁时。中风是最常见的表现体征（3例患者），其次是癫痫发作（2例患者）。到33个月时，5例患者中有4例接受了脑血管重建手术，以阻止烟雾综合征的临床进展。在结构性先天性心脏病患者的癫痫发作和卒中的鉴别诊断中，烟雾综合征应被考虑。闭塞性脑血管病的及时诊断和手术治疗可以改善这些患者的神经功能。
Objective. To describe the association between moyamoya syndrome and congenital heart disease and to discuss its clinical implications.Study Design. Retrospective analysis of a case series from two institutions.Results. Five patients with moyamoya syndrome and structural congenital heart disease were identified. Coarctation of the aorta was present in 3 patients, in association with a ventricular septal defect (1 patient), aortic and mitral valve stenoses (1 patient), and tetralogy of Fallot (1 patient). Tetralogy of Fallot and a large paramembranous ventricular septal defect were found in the other 2 patients. Four patients underwent surgical repair of their congenital heart disease during 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 halt the clinical progression of moyamoya syndrome.Conclusions. Moyamoya syndrome should be considered in the differential diagnosis of seizures and stroke in patients with structural congenital heart disease. Prompt diagnosis and surgical management of the occlusive cerebral angiopathy should lead to improved neurological outcome in these patients.