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一例罕见的诊断为主动脉瓣缺如、肺动脉瓣严重发育不全合并右心室双出口的病例报告。

A rare case of diagnosed absent aortic valve and severely hypoplastic pulmonary valve with double outlet right ventricle: A case report.

作者信息

Suvorov V V, Fedotova E P, Zaitsev V V, Dolgova E V, Popova L L, Glazunova A E, Novak M U, Nasyrov R A

机构信息

Department of Surgical Diseases of Children, Saint Petersburg State Pediatric Medical University, Saint-Petersburg, 194100, Russia.

Department of Pathological Anatomy, Saint Petersburg State Pediatric Medical University, Saint-Petersburg, 194100, Russia.

出版信息

Heliyon. 2023 Jun 20;9(6):e17373. doi: 10.1016/j.heliyon.2023.e17373. eCollection 2023 Jun.

Abstract

Agenesis of the aortic and pulmonary valves is a very rare congenital malformation of the semilunar valves. The literature describes no more than thirty cases of such anomaly in combination with congenital heart disease. Most descriptions include aplasia of either the aortic or pulmonic valve. The combination of such defect in both valves has been described in a much smaller number of scientific papers. In this article, we present a clinical case of the treatment of a patient with agenesis of aortic valve and severely hypoplastic pulmonary valve. As a result circulatory arrest occurred immediately after birth, which required the implementation of cardiopulmonary resuscitation. The child was resuscitated and transferred to the intensive care unit for further examination and treatment.

摘要

主动脉瓣和肺动脉瓣发育不全是一种非常罕见的半月瓣先天性畸形。文献中描述的这种异常合并先天性心脏病的病例不超过30例。大多数描述包括主动脉瓣或肺动脉瓣发育不全。两个瓣膜都存在这种缺陷的情况在较少的科学论文中有描述。在本文中,我们介绍了一例主动脉瓣发育不全和严重发育不良的肺动脉瓣患者的治疗临床病例。结果,患儿出生后立即发生循环骤停,需要进行心肺复苏。患儿复苏成功后被转至重症监护病房进行进一步检查和治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3994/10333606/87079d359741/gr1.jpg

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