Long-term durability of a reimplantation valve-sparing aortic root replacement can be expected in both Marfan syndrome and Loeys-Dietz syndrome
Long-term durability of a reimplantation valve-sparing aortic root replacement can be expected in both Marfan syndrome and Loeys-Dietz syndrome
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对于马凡综合征和 Loeys-Dietz 综合征，再植入保留瓣膜的主动脉根部置换术有望实现长期耐用性
DOI：
10.1093/ejcts/ezac050
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发表时间：
2022
影响因子：
3.4
通讯作者：
Matsuda Hitoshi
中科院分区：
文献类型：
--
作者：
Seike Yoshimasa;Yokawa Koki;Koizumi Shigeki;Masada Kenta;Inoue Yosuke;Morisaki Hiroko;Morisaki Takayuki;Sasaki Hiroaki;Matsuda Hitoshi
目的 本研究的目的是评估使用再植入技术对马凡综合征 (MFS) 和 Loeys-Dietz 综合征 (LDS) 患者进行保留瓣膜根部置换术的手术结果。 从 1988 年到 2020 年，研究人员对转化生长因子 - β 受体和 2、SMAD3 和转化生长因子 β - 2 进行了研究。 结果 44 名 (42.7%) 名 MFS 患者 [26 名男性，31 (7.6) 岁] 和 10 名 (35.7%) 名 Loeys-Dietz 综合征 (LDS) 患者 [7 名男性，22（标准差：8.6）岁] 没有出现任何症状。主动脉夹层并接受 包括保留瓣膜的根部置换术。术前窦直径 [MFS 患者为 46 (45–50.5) mm vs LDS 患者为 48 (47–50) mm，p= 0.420] 和主动脉瓣关闭不全 > 2+ 级的百分比 [MFS 患者为 31.8% (10/44) vs LDS 患者为 10.0% (1/10)，p= 0.667]表明 2 者之间没有显着差异 组。两组之间大于 1 级的主动脉瓣关闭不全 (p= 0.588) 和主动脉瓣再次手术 (p= 0.310) 的累积发生率相当。与 MFS 患者相比，LDS 患者初次手术后发生主动脉夹层的倾向较高 (p= 0.061)，并且主动脉再次手术的累积发生率显着较高 (p= 0.003)。 结论 MFS 和 LDS 患者的复发性主动脉瓣关闭不全和主动脉瓣再手术的累积发生率相似。与 MFS 患者相比，LDS 患者主动脉再次手术的累积发生率更高，并且初次手术后发生主动脉夹层的倾向更大。
OBJECTIVESThe goal of this study was to evaluate the surgical outcomes of a valve-sparing root replacement using the reimplantation technique for annuloaortic ectasia in patients with Marfan syndrome (MFS) and in those with Loeys-Dietz syndrome (LDS).METHODSWe reviewed 103 patients with MSF with mutations in thefibrillin-1gene and 28 patients with LDS with mutations in thetransforming growth factor-beta receptor and 2, SMAD3andtransforming growth factor beta-2from 1988 to 2020.RESULTSForty-four (42.7%) patients with MFS [26 men, 31 (7.6) years] and 10 (35.7%) patients with Loeys-Dietz syndrome (LDS) [7 men, 22 (standard deviation: 8.6) years] who had no aortic dissection and underwent valve-sparing root replacement were included. The preoperative sinus diameter [46 (45–50.5) mm in those with MFS vs 48 (47–50) mm in those with LDS,p= 0.420] and the percentage of aortic insufficiency > grade 2+ [31.8% (10/44) in patients with MFS vs 10.0% (1/10) in those with LDS,p= 0.667] revealed no significant differences between the 2 groups. The cumulative incidences of aortic insufficiency greater than grade 1 (p= 0.588) and aortic valve reoperation (p= 0.310) were comparable between the 2 groups. Patients with LDS had a higher tendency towards aortic dissection after the initial operation (p= 0.061) and a significantly higher cumulative incidence of aortic reoperation (p= 0.003) versus those with MFS.CONCLUSIONSPatients with MFS and those with LDS showed similar cumulative incidences of recurrent aortic valve insufficiency and aortic valve reoperation. Those with LDS revealed a higher cumulative incidence of aortic reoperation and a greater tendency towards aortic dissection after the initial operation compared with those with MFS.