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1型大动脉右位转位伴反向差异性发绀1例

A Case of Dextrotransposition of the Great Arteries Type I with Reversed Differential Cyanosis.

作者信息

Morimoto Yuichi, Inamura Noboru, Takemura Tukasa

机构信息

Department of Pediatrics, Kindai University Faculty of Medicine, Oosakasayamashi, Oosaka, Japan.

出版信息

AJP Rep. 2019 Jul;9(3):e235-e237. doi: 10.1055/s-0039-1692421. Epub 2019 Jul 24.

Abstract

Transposition of the great arteries type I is a severe congenital heart disease that induces serious cyanosis immediately after birth and death within 24 hours, unless proper treatment is administered. A few cases have presented with reversed differential cyanosis, manifesting as separated cyanosis with high SpO values in the lower limbs. However, there have been few reports of survivors of transposition of the great arteries type I presenting with reversed differential cyanosis. We experienced a case of transposition of the great arteries type I presenting with reversed differential cyanosis immediately after birth. The infant was urgently transported because of postnatal SpO of 40% in the upper limbs and 90% in the lower limbs. The echocardiographic diagnosis was transposition of the great arteries type I with a narrow foramen ovale. We immediately performed balloon atrioseptostomy, and the reversed differential cyanosis was improved. The infant seems to have presented with reversed differential cyanosis because of the foramen ovale narrowing and complicating pulmonary hypertension due to fetal circulation and characteristic of transposition of the great arteries type I. Congenital heart disease, presenting with reversed differential cyanosis, is a clinical condition requiring emergency management.

摘要

I型大动脉转位是一种严重的先天性心脏病,出生后立即会导致严重的青紫,并在24小时内死亡,除非进行适当的治疗。少数病例出现反向差异性青紫,表现为下肢SpO值较高的分离性青紫。然而,关于I型大动脉转位幸存者出现反向差异性青紫的报道很少。我们遇到了一例出生后立即出现反向差异性青紫的I型大动脉转位病例。由于出生后上肢SpO为40%,下肢为90%,该婴儿被紧急转运。超声心动图诊断为I型大动脉转位伴卵圆孔狭窄。我们立即进行了球囊房间隔造口术,反向差异性青紫得到改善。该婴儿出现反向差异性青紫似乎是由于卵圆孔狭窄以及胎儿循环和I型大动脉转位的特征导致的肺动脉高压并发症。先天性心脏病伴反向差异性青紫是一种需要紧急处理的临床情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/6656569/ad971c0a3b23/10-1055-s-0039-1692421-i190012-1.jpg

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