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中华诊断学电子杂志 ›› 2019, Vol. 07 ›› Issue (01) : 37 -41. doi: 10.3877/cma.j.issn.2095-655X.2019.01.008

所属专题: 文献

临床研究

肺动脉瓣缺失伴右心发育不良的诊断学特征及文献复习
杨帆1, 赵胜1, 陈欣林1,()   
  1. 1. 430070 武汉,湖北省妇幼保健院超声科
  • 收稿日期:2018-10-29 出版日期:2019-02-26
  • 通信作者: 陈欣林
  • 基金资助:
    湖北省出生缺陷影像、基因数据库的建立及应用研究(WJ2018H0132)

Diagnostic features and literature review of absence of pulmonary valve with right ventricular hypoplasia

Fan Yang1, Sheng Zhao1, Xinlin Chen1,()   

  1. 1. Department of Ultrasound, Hubei Maternal and Child Health Hospital, WuHan 430070, China
  • Received:2018-10-29 Published:2019-02-26
  • Corresponding author: Xinlin Chen
  • About author:
    Corresponding author: Chen Xinlin, Email:
引用本文:

杨帆, 赵胜, 陈欣林. 肺动脉瓣缺失伴右心发育不良的诊断学特征及文献复习[J]. 中华诊断学电子杂志, 2019, 07(01): 37-41.

Fan Yang, Sheng Zhao, Xinlin Chen. Diagnostic features and literature review of absence of pulmonary valve with right ventricular hypoplasia[J]. Chinese Journal of Diagnostics(Electronic Edition), 2019, 07(01): 37-41.

目的

探讨肺动脉瓣缺失、三尖瓣闭锁、室间隔完整、动脉导管未闭、动脉导管未闭并发右室发育不良患者的超声影像学及病理特征。

方法

回顾性分析1例于2016年2月23日就诊于湖北省妇幼保健院,在超声科经产前超声诊断为肺动脉瓣缺失病例的声像图特征和病理结果,并文献复习。

结果

孕妇27岁,孕24.6周,胎儿产前超声表现为:肺动脉瓣缺失,肺动脉与动脉导管均显示双向的血流信号,并记录到双向动脉频谱,静脉导管A波反向,三尖瓣闭锁,右心室小。诊断:法洛四联症(肺动脉瓣缺失型),右心发育不良。患儿大体检查结果:心房正位,心室右襻,大动脉正常位置,动脉导管可见,肺动脉瓣缺失,三尖瓣闭锁,右心室发育不良,室间隔完整。镜下结构:右心室及右心室游离壁可见疏松的结缔组织,部分围成血窦结构,其间可见少量散在的心肌细胞。左心室:正常心肌结构。病理诊断:肺动脉瓣缺失,三尖瓣闭锁,室间隔完整。

结论

法洛四联症合并肺动脉瓣缺失综合征同时伴有三尖瓣闭锁、室间隔完整和右心室发育不良非常罕见,掌握特殊的解剖、组织学和血液动力学特征,对本类患者的正确诊断有指导价值。

Objective

To investigate the diagnostic characteristics of ultrasound and pathology on a case with pulmonary valve absence, tricuspid atresia, intact ventricular septum, patent ductus arteriosus and right ventricular dysplasia.

Methods

The ultrasonographic features and pathological findings of one case with pulmonary valve absence diagnosed prenatally in the ultrasound department, Hubei Maternal and Child Health Hospital on February 23, 2016 were retrospectively analyzed, and the literatures were reviewed.

Results

Prenatal ultrasonography was performed at the maternal age of 27 and gestational age of 24.6W. Pulmonary valve absence was detected prenatally by ultrasound, bidirectional blood flow signals were detected at pulmonary valve and catheter, as well as bidirectional arterial spectrum and reversed venous catheter A wave. And the tricuspid valve was locked and the right ventricle was small. Diagnosis: tetralogy of Fallot (absence of pulmonary valve), right heart dysplasia. Pathological results: the heart, [S, D, S], [atrial orthotropy, right ventricular loop, normal position of the great artery], ductus arteriosus, absence of pulmonary valve, tricuspid atresia, right ventricular dysplasia. Microscopic structure: in free wall of right ventricle and right ventricle, loose connective tissue could be seen, part of which was surrounded by sinus structure, and a small amount of scattered cardiomyocytes could be seen. Left ventricular: normal myocardial structure. Pathological diagnosis: absence of pulmonary valve, tricuspid atresia and complete ventricular septum.

Conclusions

Tetralogy of Fallot with pulmonary valve absence, tricuspid atresia, complete ventricular septum and right ventricular dysplasia is rare . Mastering special anatomical, histological and hemodynamic characteristics is of guiding value for the correct diagnosis of this type of case.

图1 胎儿四腔心切面超声图像
图2 胎儿肺动脉长轴彩色多普勒超声图像
图3 胎儿肺动脉长轴彩色多普勒超声图像
图4 胎儿右心室流出道超声图像
图5 患儿心脏大体解剖图
图6 患儿心室及心室游离壁病理检查图像
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