室间隔完整的肺动脉闭锁合并先天性矫正型大动脉转位。

Pulmonary atresia with intact ventricular septum associated with congenitally corrected transposition.

作者信息

Knight C S, Litovsky S H, Pearce F B, Faye-Petersen O

机构信息

Division of Anatomic Pathology, Department of Pathology, University of Alabama at Birmingham, North Pavilion 3547, 1802 6th Avenue South, Birmingham, AL 34249, USA.

出版信息

Pediatr Cardiol. 2006 Nov-Dec;27(6):741-5. doi: 10.1007/s00246-005-1195-x.

DOI:10.1007/s00246-005-1195-x

PMID:17091327

Abstract

We present a rare case of pulmonary atresia with intact septum associated with congenitally corrected transposition. The patient was a nondysmorphic female infant. Despite a right Blalock-Taussig shunt on day 13 of life and a balloon atrial septostomy on day 23, she remained ventilator dependent with poor systemic ventricular function. The patient died at the age of 160 days, and postmortem findings are presented. The literature on this condition is reviewed, and the possibility that the left-sided morphologic right ventricle could not sustain the systemic circulation is discussed.

摘要

我们报告一例罕见的伴有完整室间隔的肺动脉闭锁合并先天性矫正型大动脉转位病例。患者为一名非畸形女婴。尽管在出生后第13天行右Blalock-Taussig分流术，第23天行球囊房间隔造口术，但她仍依赖呼吸机，体循环心室功能差。患者于160天时死亡，并呈现尸检结果。本文回顾了关于这种疾病的文献，并讨论了左侧形态学右心室无法维持体循环的可能性。