关键词: 马凡综合征, 主动脉瘤, 主动脉夹层

Abstract:

Objective:To summarize the surgical experiences in Marfan syndrome accompanied with descending aortic aneurysms. Methods:The clinical data of 16 cases of Marfan syndrome accompanied with descending aortic aneurysms admitted between 1998 and 2005 in Shanghai Chest Hospital Affiliated to Shanghai Jiao Tong University were analyzed retrospectively. Results:All the 16 cases had history of Marfan syndrome and 2 of them had an additional experience of Bentall operation.All the 16 cases underwent operations,including traditional operation in 12,fenestration in 2 and endovascular treatment in 2.Two cases died postoperatively and 1 of them died on the 10th day after the endovascular therapy.Thirteen cases were followed up for 4 months to 7 years,of which,1 died of rupture of the thoracic aortic dissection suddenly,and 2 were excellent.Six of 10 cases with hypertension preoperatively showed normal blood pressure,and the other 4 were controlled by medicine. Conclusion:The aorta wall structures of the aneurysms accompanied with Marfan syndrome are more fragile than the normal patients and though a Bentall operation advanced.There is even likely recurrence of dissections and aneurysms.It could be treated with traditional operation primarily and the indication for the endovascular technique should be further discussed

Key words: Marfan syndrome, aortic aneurysms, aortic dissection