Division of Cardiology, Department of Radiology, St Paul's Hospital, University of British Columbia, Vancouver, BC, Canada.
J Thorac Imaging. 2012 Jul;27(4):W88-90. doi: 10.1097/RTI.0b013e31821424de.
Marfan syndrome is associated with a high incidence of aortic root aneurysm and life-threatening aortic dissection. With the successful use of surgical aortic root replacement, dissection-related mortality has been significantly reduced. We present the case of a patient with Marfan syndrome who presented with heart failure secondary to an unusual graft-related complication 14 years after a Bentall procedure. Investigations revealed a supra-aortic stenosis resulting from a kink in the Bentall graft caused by pressure from an expanding aortic arch aneurysm. The patient underwent surgery with improvement in his ejection fraction and heart failure symptoms.
马凡综合征常并发主动脉根部瘤，且易发生致死性的主动脉夹层。得益于主动脉根部置换术的成功应用，与夹层相关的死亡率已显著降低。我们报告了 1 例马凡综合征患者，该患者在 Bentall 手术后 14 年因罕见的移植物相关并发症导致心力衰竭。检查发现，升主动脉瘤扩张导致 Bentall 移植物受压，出现扭曲，进而导致主动脉弓上狭窄。患者接受了手术治疗，射血分数和心力衰竭症状均得到改善。
