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马凡综合征患者主动脉根部手术的长期结果:单中心经验

Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

作者信息

Nicolo Francesco, Romeo Francesco, Lio Antonio, Bovio Emanuele, Scafuri Antonio, Bassano Carlo, Polisca Patrizio, Pellegrino Antonio, Nardi Paolo, Chiariello Luigi, Ruvolo Giovanni

机构信息

Cardiac Surgery Unit and Marfan Center, Tor Vergata University Policlinic, Rome, Italy. Electronic correspondence:

Department of Cardiovascular Disease, Tor Vergata University Policlinic, Rome, Italy.

出版信息

J Heart Valve Dis. 2017 Jul;26(4):397-404.

Abstract

BACKGROUND AND AIM OF THE STUDY

The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation.

METHODS

Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29).

RESULTS

No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33).

CONCLUSIONS

The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

摘要

研究背景与目的

本研究旨在比较接受Bentall或David手术进行主动脉根部置换的马方综合征(MFS)主动脉根部疾病患者的长期疗效。

方法

自1994年以来,作者所在的马方综合征中心共随访了59例患者,这些患者分别接受了Bentall手术(Bentall组,n = 30)或David手术(David组,n = 29)。

结果

无手术死亡记录。20年后(平均随访97±82个月;范围1至369个月),未观察到与人工瓣膜相关的严重出血或血栓栓塞事件;Bentall组的20年生存率为94±6%,David组为100%(p = 0.32)。Bentall组主动脉瓣功能障碍无需再次干预的比例为100%,David组为75±13%(p = 0.04)。这种组间差异在随访的前八年之后变得显著,并且主要与一种特定的家族遗传表型有关,四名再次手术的患者中有三名涉及该表型。Bentall组全因死亡、心肌梗死、中风、人工瓣膜相关并发症以及主动脉任何节段再次干预的无事件生存率为69±12%,David组为67±14%(p = 0.33)。

结论

Bentall手术和David手术在MFS患者中均具有令人满意的长期疗效。人工瓣膜相关并发症的低发生率表明Bentall手术仍将是标准的外科治疗方法。用于主动脉扩张程度较小的主动脉再植技术,再次手术率较低。对于患有严重家族遗传表型的患者,应仔细关注主动脉再植技术。

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