Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.
Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.
复制标题
患有圆锥干异常的成人进行性主动脉瘤和夹层的患病率和风险。
DOI：
10.1093/ehjci/jeab273
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发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Connolly,HeidiM
中科院分区：
文献类型：
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作者：
Egbe,AlexanderC;Miranda,WilliamR;Bonnichsen,CrystalR;Jain,CCharles;Crestanello,JuanA;Francois,Christopher;Katta,RenukaR;Iftikhar,Momina;Goda,AhmedY;Andi,Kartik;Gandhi,Sangeetha;Connolly,HeidiM
圆锥动脉干异常具有流出道和大动脉的共同胚胎发育缺陷，导致易患主动脉瘤。本研究的目的是描述的患病率和进行性主动脉瘤的成年人conotrumal anomalies.Methods和resultsRetrospective研究成年人conotrumal异常，进行横断面成像2003-20的风险。动脉瘤定义为主动脉根部/升主动脉中段>2.1 mm/m2/>1.9 mm/m2，进展性动脉瘤定义为增大>2 mm，严重动脉瘤定义为尺寸>50 mm。在2261例患者（38 ± 12岁;男性58%）中，1167例（52%）患有主动脉瘤，205例（14%）患有严重主动脉瘤。主动脉根部/升主动脉中段的平均年增长为0.3 ± 0.1 mm/0.2 ± 0.1 mm。进行性主动脉瘤的3年、5年和7年累积发生率分别为4%、7%和9%。动脉瘤生长率随年龄增长而下降，40岁后无显著增长。横截面成像和超声心动图的主动脉指数之间有很好的相关性。在950例女性中，184例妊娠≥1次，81例（44%）患者在妊娠前患有主动脉瘤。妊娠期间未发生主动脉夹层或主动脉瘤进展。总的来说，在7984 patient-years of following.ConclusionsAortic动脉瘤是常见的圆锥动脉干异常的患者中，没有主动脉夹层。然而，进展性动脉瘤或夹层的风险较低。总的来说，这些数据表明一个良性的自然史，也许是一个不太频繁的需要横断面成像。需要进一步的研究来确定该人群手术干预的最佳时机。
AimsConotruncal anomalies share common embryogenic defects of the outflow tracts and great arteries, which result in a predisposition to aortic aneurysms. The purpose of this study was to describe the prevalence and risk of progressive aortic aneurysms in adults with conotruncal anomalies.Methods and resultsRetrospective study of adults with conotruncal anomalies that underwent cross-sectional imaging 2003–20. Aneurysm was defined as aortic root/mid-ascending aorta >2.1 mm/m2/>1.9 mm/m2, progressive aneurysm as increase by >2 mm, and severe aneurysm as dimension >50 mm. Of 2261 patients (38 ± 12 years; male 58%), 1167 (52%) had an aortic aneurysm, and 205 (14%) had a severe aortic aneurysm. Mean annual increase in aortic root/mid-ascending aorta was 0.3 ± 0.1 mm/0.2 ± 0.1 mm. The 3-, 5-, and 7-year cumulative incidence of the progressive aortic aneurysm was 4%, 7%, and 9%, respectively. The rate of aneurysm growth decreased with age, with no significant growth after age 40 years. There was an excellent correlation between aortic indices from cross-sectional imaging and echocardiography. Of 950 females, 184 had ≥1 pregnancy, and 81 (44%) of the 184 patients had aortic aneurysm prior to pregnancy. There was no aortic dissection or progression of the aortic aneurysm during pregnancy. Overall, there was no aortic dissection during 7984 patient-years of follow-up.ConclusionsAortic aneurysm was common in patients with conotruncal anomalies. However, the risk of progressive aneurysm or dissection was low. Collectively, these data suggest a benign natural history and perhaps a less frequent need for cross-sectional imaging. Further studies are required to determine the optimal timing for surgical intervention in this population.