Schoenhoff Florian S, Cameron Duke E, Matyas Gabor, Carrel Thierry P
Department of Cardiovascular Surgery, University Hospital Bern, Freiburgstrasse, 3010 Bern, Switzerland.
Future Cardiol. 2011 Jul;7(4):557-69. doi: 10.2217/fca.11.37.
Acute dissection and rupture of aortic aneurysms comprise for 1-2% of all deaths in industrialized countries. Dilation of the aorta is caused by a multitude of mechanisms including inherited connective tissue disorders such as Marfan syndrome (MFS). MFS is one of the most common inherited connective tissue disorders affecting 1 in 5000 individuals. Although the phenotype of MFS can be quite variable, aneurysmal dilation of the aortic root and consecutive acute aortic dissection is the leading cause of death in this patient population. Over the past years it has been shown that a comprehensive understanding of this disorder provides greater understanding of vascular wall biology and identifies pathways relevant to aortic aneurysms and dissection in general. The current review discusses the surgical management of patients with MFS with a special emphasis on indications for surgery in this complex group of patients.
在工业化国家，急性主动脉瘤夹层和破裂占所有死亡病例的1%至2%。主动脉扩张由多种机制引起，包括遗传性结缔组织疾病，如马凡综合征（MFS）。MFS是最常见的遗传性结缔组织疾病之一，每5000人中就有1人受影响。尽管MFS的表型差异很大，但主动脉根部瘤样扩张及随后的急性主动脉夹层是该患者群体的主要死因。在过去几年中，已表明对这种疾病的全面了解能增进对血管壁生物学的认识，并确定与主动脉瘤和夹层总体相关的途径。本综述讨论了MFS患者的手术治疗，特别强调了这一复杂患者群体的手术指征。
