Song Howard K, Preiss Liliana R, Maslen Cheryl L, Kroner Barbara, Devereux Richard B, Roman Mary J, Holmes Kathryn W, Tolunay H Eser, Desvigne-Nickens Patrice, Asch Federico M, Milewski Rita K, Bavaria Joseph, LeMaire Scott A
J Heart Valve Dis. 2014 May;23(3):292-8.
The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC).
At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR).
AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less.
AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.
马凡综合征（MFS）患者行保留主动脉瓣（AVS）的主动脉根部置换术的长期疗效仍不明确。本研究旨在确定参与遗传性触发胸主动脉瘤和心血管疾病注册研究（GenTAC）的MFS患者中AVS根部置换术的应用情况及疗效。
在本次分析时，788例MFS患者纳入GenTAC注册研究，其中288例接受了主动脉根部置换术。将接受AVS手术的患者与接受主动脉瓣置换术（AVR）的患者进行比较。
43.5%的MFS患者接受了AVS根部置换术，且在过去五年中AVS的应用频率有所增加。与AVR患者相比，AVS患者手术时年龄更小（31.0岁对36.3岁，p = 0.006），更有可能接受择期而非急诊手术，AVR患者中主动脉瓣功能障碍和主动脉夹层更可能是手术的主要指征。平均随访6.2±3.6年后，87例AVS患者均未需要再次手术；相比之下，平均随访10.5±7.6年后，11.5%的AVR患者需要进行主动脉根部再次手术。主动脉瓣功能持久，95.8%的AVS患者主动脉瓣关闭不全分级为轻度或更低。
AVS根部置换术在MFS患者中应用普遍，迄今为止主动脉修复和主动脉瓣功能的持久性良好。这些结果证明在择期手术中继续应用该手术是合理的。GenTAC注册研究将是未来评估AVS根部置换术长期持久性的有用资源。
