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新生儿患者法洛四联症合并肺动脉及肺动脉瓣狭窄的成功手术修复:一例报告

Successful surgical repair of Taussig Bing anomaly with pulmonary artery and pulmonary valve stenosis in a neonate patient: A case report.

作者信息

Barakat Lyne, Alabdullah Salam, Alezzo Adnan, Alsaffaf Yousef, Takkem Saleh

机构信息

Faculty of Medicine, Hama University, Hama, Syria.

Faculty of Medicine, Hama University, Hama, Syria.

出版信息

Int J Surg Case Rep. 2024 Dec;125:110462. doi: 10.1016/j.ijscr.2024.110462. Epub 2024 Oct 20.

Abstract

INTRODUCTION AND IMPORTANCE

Taussig Bing anomaly (TBA), a subtype of double outlet right ventricle, is a rare congenital cardiac malformation. TBA consists of double outlet right ventricle, sub-pulmonary ventricular septal defect, and side-by-side localization of the great arteries. The association of pulmonary artery and pulmonary valve stenosis with TBA is an uncommon phenomenon.

CASE PRESENTATION

A 2-day-old male neonate presented with central cyanosis and poor breastfeeding following birth. His mother had no history of drug use or medical issues during pregnancy. The neonate was diagnosed TBA with pulmonary artery and pulmonary valve stenosis based on investigations including electrocardiogram, chest X-rays, and echocardiography. At three months of age, the child underwent palliative Blalock-Taussig shunt surgery (BTS) before undergoing Réparation à l'Ètage Ventriculaire (REV) repair surgery at the age of two. The follow-up showed that the child is currently in good health without any complications.

CLINICAL DISCUSSION

Patients with TBA should be diagnosed early to plan the correct surgical technique and timely intervention, which can significantly improve the quality of life for these patients.

CONCLUSION

TBA is a surgical challenge, which becomes even more challenging when accompanied by other malformations such as pulmonary artery and pulmonary valve stenosis. Medical team should work together to improve the patient's outcome.

摘要

引言与重要性

陶西格-宾畸形(TBA)是双出口右心室的一种亚型,是一种罕见的先天性心脏畸形。TBA包括双出口右心室、肺动脉下室间隔缺损以及大动脉并列定位。肺动脉和肺动脉瓣狭窄与TBA相关是一种不常见的现象。

病例介绍

一名2日龄男婴出生后出现中心性青紫和母乳喂养困难。其母亲在孕期无吸毒史或医疗问题。基于心电图、胸部X线和超声心动图等检查,该新生儿被诊断为患有TBA合并肺动脉和肺动脉瓣狭窄。3个月大时,该患儿接受了姑息性布劳洛克-陶西格分流术(BTS),并于2岁时接受了心室修复术(REV)。随访显示该患儿目前健康状况良好,无任何并发症。

临床讨论

TBA患者应尽早诊断,以规划正确的手术技术并及时干预,这可显著提高这些患者的生活质量。

结论

TBA是一项手术挑战,当伴有肺动脉和肺动脉瓣狭窄等其他畸形时,挑战会更大。医疗团队应共同努力改善患者的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4d3/11550062/f1963e4667ad/gr2.jpg

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