PDF(Pubmed)
Abstract：
A 16-year-old girl with Loeys-Dietz syndrome presented with an acute, complicated type B aortic dissection (AD) with mesenteric and right renal malperfusion owing to a dynamic obstruction. The anatomy of her AD and her genetic aortography were suboptimal for thoracic endovascular aortic repair. Given the concern for anticipated late aortic degeneration and the need for open aortic repair, she underwent successful transfemoral endovascular septal fenestration with stenting of the fenestration into the superior mesenteric artery and additional stenting of the right renal artery. Her renal failure and mesenteric angina resolved, and she was discharged home. Endovascular fenestration provides an elegant solution for AD-associated dynamic malperfusion of aortic branch vessels without compromising future open aortic repairs.
摘要：
一名患有Loeys-Dietz综合征的16岁女孩出现急性，复杂的B型主动脉夹层（AD）与肠系膜和右肾灌注不良由于动态梗阻。她的AD的解剖结构和遗传主动脉造影对于胸主动脉腔内修复是次优的。考虑到预期的晚期主动脉变性和开放主动脉修复的需要，她接受了成功的经股动脉腔内间隔开窗术，并将开窗支架置入肠系膜上动脉，并额外置入右肾动脉。她的肾衰竭和肠系膜心绞痛缓解了,她出院回家了.血管内开窗术为AD相关的主动脉分支血管动态灌注不良提供了一种优雅的解决方案，而不会影响未来的开放式主动脉修复。
