Toolan Caroline, Oo Shwe, Shaw Matthew, Field Mark, Kuduvalli Manoj, Harrington Deborah, Nawaytou Omar
Department of Cardiac Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK.
Eur J Cardiothorac Surg. 2021 Nov 19. doi: 10.1093/ejcts/ezab491.
Pre-emptive strategies to manage the aortic complications of Marfan syndrome have resulted in improved life expectancy yet, secondary to the variation of phenotypic expression, anticipating the risk and nature of future aortic events is challenging. We examine rates of new aortic events and reinterventions in a Marfan cohort following initial aortic presentation.
Retrospective cohort study of Marfan patients with aortic pathology presenting to our institution 1998-2018. Patients were grouped according to index event: aortic dissection or root aneurysm. Patients with aortic dissection were classified according to Debakey criteria. Incidence of new aortic events and frequency of reintervention were analysed.
One hundred and twenty-six aortic procedures were performed in 74 Marfan patients with a median follow-up of 7 years. Forty-seven patients had an index event of root aneurysm and 27 had aortic dissection. Following operative intervention in the aneurysm group, 7 patients developed Debakey III dissections raising the overall number of patients who developed dissection within this cohort to 34. Reinterventions were more frequent in the dissection group with full replacement of the native aorta in 5 patients.
After operative intervention on the proximal aorta, a proportion will develop distal pathology. A greater focus on factors contributing to future events, such as mapping genotypes to clinical course, may lead the way for targeted operative techniques and surveillance.
用于处理马凡综合征主动脉并发症的预防性策略已使预期寿命得到提高，然而，由于表型表达的差异，预测未来主动脉事件的风险和性质具有挑战性。我们研究了马凡综合征队列初次出现主动脉病变后新的主动脉事件发生率和再次干预情况。
对1998年至2018年在我们机构就诊的患有主动脉病变的马凡综合征患者进行回顾性队列研究。患者根据索引事件分组：主动脉夹层或根部动脉瘤。主动脉夹层患者根据德巴基标准进行分类。分析新的主动脉事件发生率和再次干预频率。
74例马凡综合征患者共进行了126次主动脉手术，中位随访时间为7年。47例患者的索引事件为根部动脉瘤，27例为主动脉夹层。在动脉瘤组进行手术干预后，7例患者发生德巴基III型夹层，使该队列中发生夹层的患者总数增至34例。夹层组的再次干预更为频繁，5例患者进行了主动脉全置换。
在对升主动脉进行手术干预后，一部分患者会出现远端病变。更加关注导致未来事件的因素，如将基因型与临床病程对应，可能会为有针对性的手术技术和监测指明方向。
