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永存动脉干

Authors
Brian Soriano, MD
David R Fulton, MD
Section Editor
John K Triedman, MD
Deputy Editor
Carrie Armsby, MD, MPH
Translators
刘斌, 主任医师，教授

引言

永存动脉干(永存动脉干,TA)也被称为共同动脉干，是一种紫绀型先天性心脏缺陷。在这种疾病中，血液从心脏通过单一动脉干瓣膜被泵入动脉干，动脉干会发出主动脉和肺动脉(图 1)。

本文将总结永存动脉干的解剖学、临床表现、诊断以及治疗。

流行病学

据报道，永存动脉干在活产儿中的发病率为0.06‰-0.1‰[1,2]。虽然只有0.7%的所有先天性心脏病变是由于永存动脉干，但它占了所有危重先天性心脏病病例的4%[1]。

胚胎学

在正常心脏发育的早期阶段，大动脉起源于单一动脉干根部。到第5周胎龄末时，该根部内会形成一个壁(圆锥动脉干间隔)，将根部分隔成发育完全的主肺动脉和升主动脉。在接下来2周的发育中，圆锥(漏斗部)间隔随着圆锥动脉干间隔同心内膜垫和室间隔的融合而关闭，分隔新形成的左心室和右心室。

圆锥动脉干间隔的发育障碍可导致包括永存动脉干在内的圆锥动脉干畸形。壁发育缺陷也会阻碍形成单独的主动脉瓣和肺动脉瓣，从而产生与永存动脉干相关的单一动脉干瓣膜[3]。

                                       

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Literature review current through: 2017-06 . | This topic last updated: 2016-07-12.
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