Tian Miao, Si Ming-Sing, Zhang Yong, Ding Yu, Chen Jimei
Department of Cardiac Surgery, Guangdong Provincial People's Hospital, School of Medicine, South China University of Technology, Guangzhou, China.
Department of Cardiac Surgery, Guangdong Provincial Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.
Transl Pediatr. 2020 Oct;9(5):695-701. doi: 10.21037/tp-20-146.
Aortic complications of Loeys-Dietz syndrome (LDS) rarely present in children. Here we describe a case of LDS type 2 in a 3-year-old child with severe aortic root dilation and severe aortic regurgitation. A Bentall procedure combined with a Cabrol-type coronary modification was used to treat this child. In order to minimize the need for reintervention as the child grows. We chose a composite valve-graft by a St Jude Regent 21# mechanical valve seated within a 24 mm Gore-Tex graft to finish the Bentall procedure. Echocardiographic studies demonstrated good valve and ventricular function at 1-year follow-up. This child is one of the youngest LDS patients to receive a Bentall procedure and the way using a composite valve-graft to finish the operation can provide a reference for the surgical strategies of such patients in the future. Children with LDS and severe aortic annulus dilatation combined with severe aortic valve regurgitation require early surgical intervention, and implantation of a mechanical valved-conduit can be utilized successfully. Life-long follow-up of the valved-conduit and arterial vessels of these patients is necessary. The experience gained from this case contributes to the management of the rare LDS patient who presents at an early age with aortic root and valve pathology. Close monitoring and early intervention are important.
洛伊斯-迪茨综合征（LDS）的主动脉并发症在儿童中很少见。在此，我们描述了一名3岁患有严重主动脉根部扩张和严重主动脉反流的2型LDS患儿。采用Bentall手术联合Cabrol型冠状动脉改良术治疗该患儿。为了尽量减少患儿长大后再次干预的需求，我们选择了一个由置于24毫米戈尔特斯（Gore-Tex）人工血管内的圣犹达Regent 21#机械瓣膜组成的复合瓣膜移植物来完成Bentall手术。超声心动图研究显示，随访1年时瓣膜和心室功能良好。该患儿是接受Bentall手术的最年轻的LDS患者之一，使用复合瓣膜移植物完成手术的方式可为未来此类患者的手术策略提供参考。患有LDS且严重主动脉瓣环扩张合并严重主动脉瓣反流的儿童需要早期手术干预，植入带机械瓣膜的管道可成功应用。对这些患者的带瓣膜管道和动脉血管进行终身随访是必要的。该病例所获得的经验有助于管理罕见的、在幼年时出现主动脉根部和瓣膜病变的LDS患者。密切监测和早期干预很重要。
