背景:心脏手术中医源性主动脉夹层是一种罕见但严重的并发症。目前,没有制定预防策略。我们在此报告了一例无主动脉疾病家族史的III型Loeys-Dietz综合征老年患者在主动脉根置换期间发生术中主动脉夹层的病例。
方法:一名60岁男性因StanfordB型急性主动脉夹层入院治疗。他被发现有主动脉根部扩张和严重的主动脉瓣反流。因此,进行了选择性Bentall手术。术后计算机断层扫描显示新的StanfordA型主动脉夹层,可能是由于手术期间的主动脉插管而形成的。患者行保守治疗,于术后第34天顺利出院。尽管他没有主动脉疾病的家族史,基因测试显示未报告的SMAD3移码突变(c.742_749dup,p.Gln252ThrfsTer7),患者被诊断为III型Loeys-Dietz综合征。
结论:在患有结缔组织疾病的患者中,主动脉操作可能成为严重并发症的原因。避免使用侵入性技术,如插管和交叉夹紧,实施治疗策略,如主动脉以外的其他部位的灌注和开放远端吻合,可以预防这些并发症,可能是有用的治疗方式。即使患者年龄较大且没有主动脉疾病家族史,也应考虑结缔组织疾病的可能性。
BACKGROUND: Iatrogenic aortic dissection during cardiac surgery is a rare but critical complication. At present, no strategies have been developed to prevent it. We herein report a
case of intraoperative aortic dissection during aortic root replacement in an older patient with Loeys-Dietz syndrome type III who had no family history of aortic disease.
METHODS: A 60-year-old man was admitted to the hospital for Stanford type B acute aortic dissection and given conservative treatment. He was found to have aortic root dilatation and severe aortic regurgitation. Thus, elective Bentall procedure was performed. Postoperative computed tomography showed new Stanford type A aortic dissection that may have developed due to aortic cannulation during surgery. The patient was given conservative treatment and successfully discharged to home at postoperative day 34. Although he had no family history of aortic disease, a genetic test revealed an unreported SMAD3 frameshift mutation (c.742_749dup, p. Gln252ThrfsTer7), and the patient was diagnosed with Loeys-Dietz syndrome type III.
CONCLUSIONS: In patients with connective tissue disorder, aortic manipulations may become the cause of critical complications. Avoiding the use of invasive techniques, such as cannulation and cross-clamping, and implementing treatment strategies, such as perfusion from other sites than the aorta and open distal anastomosis, can prevent these complications, and may be useful treatment modalities. The possibility of connective tissue disease should be considered even if the patient is older and has no family history of aortic disease.