Fan Wen-Po, Li Hsing-Yuan, Tseng Szu-Yin, Juan Chien-Chang, Hwang Betau, Niu Dau-Ming, Lee Pi-Chang
Division of Pediatric cardiology, Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
Division of Pediatric, National Yang Ming Chiao Tung University Hospital, Yilan, Taiwan, ROC.
J Chin Med Assoc. 2021 May 1;84(5):540-544. doi: 10.1097/JCMA.0000000000000520.
Marfan syndrome is an inherited connective tissue disease that causes aortic root dilatation and dissection and requires surgical intervention. Apart from emergent surgery for aortic dissection or aortic aneurysmal rupture, prophylactic surgical intervention can also be administered, depending on the severity of aortic root dilatation. The direct relationship between surgical intervention and aortic regurgitation was seldom mentioned in previous studies.
A retrospective cohort study was designed to determine the clinical presentations of prophylactic surgery in patients with Marfan syndrome. Between January 2009 and May 2019, 112 patients, adolescents and young adults, treated in the Department of Pediatric Cardiology of Taipei Veterans General Hospital, were enrolled. All patients' sex, body measurements, echocardiography reports, and surgical notes were collected for statistical analysis.
Among the participants, nine patients (8%) underwent the Bentall procedure, and the other 103 did not receive surgical intervention. The operation group had a larger aortic root size (4.89 vs 2.86 cm, p < 0.001), more dilated left ventricle (4.81 vs 4.1 cm, p = 0.002), and higher prevalence of moderate and severe aortic regurgitation (66% vs 1%, p < 0.001) than the nonoperation group.
Among adolescents and young adults with Marfan syndrome, echocardiographic presentation of aortic root dilatation, left ventricular dilatation, and significant aortic regurgitation was significantly associated with prophylactic surgical intervention. According to the study, significant aortic regurgitation should also be considered as an important indication for prophylactic surgery.
马凡综合征是一种遗传性结缔组织疾病，可导致主动脉根部扩张和夹层形成，需要手术干预。除了针对主动脉夹层或主动脉瘤破裂的急诊手术外，还可根据主动脉根部扩张的严重程度进行预防性手术干预。既往研究很少提及手术干预与主动脉反流之间的直接关系。
设计一项回顾性队列研究，以确定马凡综合征患者预防性手术的临床表现。2009年1月至2019年5月，纳入台北荣民总医院小儿心脏科治疗的112例青少年和青年患者。收集所有患者的性别、身体测量数据、超声心动图报告和手术记录进行统计分析。
参与者中，9例患者（8%）接受了Bentall手术，其他103例未接受手术干预。手术组的主动脉根部尺寸更大（4.89 vs 2.86 cm，p<0.001），左心室扩张更明显（4.81 vs 4.1 cm，p = 0.002），中重度主动脉反流的患病率高于非手术组（66% vs 1%，p<0.001）。
在患有马凡综合征的青少年和青年中，主动脉根部扩张、左心室扩张和明显主动脉反流的超声心动图表现与预防性手术干预显著相关。根据该研究，明显的主动脉反流也应被视为预防性手术的重要指征。
