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与意外宫内猝死相关的动脉导管孤立性夹层

Isolated Dissection of the Ductus Arteriosus Associated with Sudden Unexpected Intrauterine Death.

作者信息

Fedrigo Marny, Visentin Silvia, Veronese Paola, Barison Ilaria, Giarraputo Alessia, Cosmi Erich, Thiene Gaetano, Gervasi Maria Teresa, Basso Cristina, Angelini Annalisa

机构信息

Department of Cardiac, Thoracic, Vascular Sciences, and Public Health, University of Padua, 35128 Padua, Italy.

Department of Woman and Children Health, University of Padua, 35128 Padua, Italy.

出版信息

J Cardiovasc Dev Dis. 2021 Jul 31;8(8):91. doi: 10.3390/jcdd8080091.

Abstract

We report five cases of sudden intrauterine death due to premature closure of the ductus arteriosus. In four cases, this was caused by dissecting the hematoma of the ductus arteriosus with intimal flap and obliteration of the lumen. In one case, the ductus arteriosus was aneurysmatic, with lumen occlusion caused by thrombus stratification. No drug therapy or free medication consumption were reported during pregnancy. The time of stillbirth ranged between 26 and 33 gestational weeks. We performed TUNEL analysis for apoptosis quantification. The dissecting features were intimal tears with flap formation in four of the cases, just above the origin of the ductus arteriosus from the pulmonary artery. The dissecting hematoma of the ductus arteriosus extended downward to the descending aorta and backward to the aortic arch with involvement of the left carotid and left subclavian arteries. TUNEL analysis showed a high number of apoptotic smooth muscle cells in the media in two cases. Abnormal ductal remodeling with absence of subintimal cushions, lacunar spaces rich in glycosaminoglycans (cystic medial necrosis), and smooth muscle cell apoptosis were the pathological substrates accounting for failure of remodeling process and dissection.

摘要

我们报告了5例因动脉导管过早关闭导致的宫内猝死病例。在4例中,这是由于动脉导管血肿伴内膜瓣形成及管腔闭塞所致。在1例中,动脉导管呈瘤样扩张,管腔因血栓分层而闭塞。孕期未报告药物治疗或服用非处方药情况。死产发生时间在妊娠26至33周之间。我们进行了TUNEL分析以定量凋亡情况。4例病例的解剖特征为内膜撕裂伴瓣形成,位于动脉导管从肺动脉起始处的上方。动脉导管的夹层血肿向下延伸至降主动脉,向后延伸至主动脉弓,累及左颈动脉和左锁骨下动脉。TUNEL分析显示2例中膜有大量凋亡的平滑肌细胞。内膜下垫缺如、富含糖胺聚糖的腔隙(囊性中膜坏死)以及平滑肌细胞凋亡等异常导管重塑是导致重塑过程失败和夹层形成的病理基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9798/8396886/02e5362855e7/jcdd-08-00091-g001.jpg

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