Berkman M, Farkas E, Tabet J, Seban C, Langlois J, Binet J P, Chiche P
Arch Mal Coeur Vaiss. 1979 Aug;72(8):831-41.
The theoretical and practical problems posed by aneurysms of the ascending aorta with mucoid infiltration of the Media and the aortic leaflets are discussed with reference to two suggestive cases. These histological changes are not specific for Marfan's syndrome, the relation with which is very inconstant. Incomplete replacement of the affected tissues risks a recurrence which is especially serious when the sinus of Valsalva is involved. Therefore, total replacement of the ascending aorta with aortic valve replacement and reimplantation of the coronary arteries is generally indicated. Histology of the excised tissues should be performed routinely with specific stains for mucopolysaccharides. Close follow-up of operated patients with this condition is recommended, especially those with only partial replacements.
结合两个具有启发性的病例,讨论了升主动脉瘤伴中膜和主动脉瓣叶黏液样浸润所带来的理论和实际问题。这些组织学改变并非马凡综合征所特有,与马凡综合征的关系非常不稳定。受累组织的不完全置换有复发风险,当累及主动脉窦时尤其严重。因此,一般建议行升主动脉全置换并置换主动脉瓣,同时重新植入冠状动脉。切除组织的组织学检查应常规使用黏多糖特异性染色。建议对患有这种疾病的手术患者进行密切随访,尤其是那些仅进行部分置换的患者。