Schievink W I, Mokri B, Piepgras D G, Gittenberger-de Groot A C
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Neurosurgery. 1996 Oct;39(4):685-9; discussion 689-90. doi: 10.1097/00006123-199610000-00006.
The number of patients with congenital heart disease who survive to adolescence and adulthood continues to increase. We review our experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with congenital heart disease, expanding the clinical spectrum of the cerebrovascular abnormalities that may be encountered in this group of patients.
All patients with congenital heart disease and intracranial aneurysms of cervicocephalic arterial dissections, who were evaluated at the Mayo Clinic between 1969 and 1992, were identified.
Congenital heart disease was diagnosed in 3 (8%) of 36 children with intracranial aneurysms, in 5 (0.3%) of 1994 adults with intracranial aneurysms, in 1 (4%) of 25 children with cervicocephalic arterial dissections, and in 5 (2%) of 250 adults with cervicocephalic arterial dissections. The mean age of the 14 patients was 32 years (range, 13-58 yr). The congenital heart disorders consisted of complex cardiac anomalies in three patients (truncus arteriosus, transposition of the great arteries, and tricuspid atresia in one patient each), pulmonic valve or arterial stenosis in two patients, aortic coarctation in four patients, and bicuspid aortic valve in five patients. Only one patient had an intracranial aneurysm and coarctation of the aorta.
Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.
存活至青春期和成年期的先天性心脏病患者数量持续增加。我们回顾了先天性心脏病患者非感染性颅内动脉瘤和头颈部动脉夹层的治疗经验，拓展了这组患者可能出现的脑血管异常的临床谱。
确定1969年至1992年间在梅奥诊所接受评估的所有先天性心脏病合并颅内动脉瘤或头颈部动脉夹层的患者。
36例颅内动脉瘤患儿中有3例（8%）诊断为先天性心脏病，1994例颅内动脉瘤成人中有5例（0.3%），25例头颈部动脉夹层患儿中有1例（4%），250例头颈部动脉夹层成人中有5例（2%）。14例患者的平均年龄为32岁（范围13 - 58岁）。先天性心脏疾病包括3例复杂心脏畸形（永存动脉干、大动脉转位和三尖瓣闭锁各1例），2例肺动脉瓣或动脉狭窄，4例主动脉缩窄，5例二叶式主动脉瓣。仅1例患者同时患有颅内动脉瘤和主动脉缩窄。
患有各种先天性心脏疾病的个体发生颅内动脉瘤和头颈部动脉夹层的风险可能增加，尤其是在青春期。头颈部的肌性动脉起源于神经嵴细胞，而神经嵴在心脏早期发育中也至关重要，这表明神经嵴异常可能是解释这种关联的共同致病因素。
