伴有左心室发育不全和左房室瓣狭窄的不完全性房室间隔缺损

Incomplete atrioventricular septal defect with hypoplastic left ventricle and left atrioventricular valve stenosis.

作者信息

Nishimura M, Yamagishi M, Fujiwara K, Yoshida M, Kitamura N

机构信息

Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan.

出版信息

Jpn J Thorac Cardiovasc Surg. 2001 Apr;49(4):247-9. doi: 10.1007/BF02913524.

DOI:10.1007/BF02913524

PMID:11355259

Abstract

A 2-month-old male infant with incomplete atrioventricular septal defect associated with a hypoplastic left ventricle and left atrioventricular valve stenosis successfully underwent biventricular repair. Echocardiography showed marked dilatation in the right ventricle and a diminutive left ventricle. However, the left ventricular chamber occupied the apex. Left atrioventricular valve stenosis was due to a solitary papillary muscle. Cardiac catheterization showed pulmonary/systemic flow ratio of 3.61, left ventricular end-diastolic volume of 63% of normal, and right ventricular end-diastolic volume of 324% of normal. During surgical repair, the solitary papillary muscle was divided longitudinally and the ostium primum was closed with a bovine pericardium. Postoperative left ventricular function was appropriate. Even when the preoperative end-diastolic left ventricular volume is small, if the left ventricle chamber is at the apex, then the left ventricular performance can be expected to be appropriate to tolerate the volume load after ostium primum closure.

摘要

一名2个月大的男性婴儿，患有不完全性房室间隔缺损，合并左心室发育不全和左房室瓣狭窄，成功接受了双心室修复术。超声心动图显示右心室明显扩张，左心室较小。然而，左心室腔占据心尖。左房室瓣狭窄是由于单一乳头肌所致。心导管检查显示肺循环/体循环血流量比值为3.61，左心室舒张末期容积为正常的63%，右心室舒张末期容积为正常的324%。在手术修复过程中，将单一乳头肌纵向分开，用牛心包关闭原发孔。术后左心室功能正常。即使术前左心室舒张末期容积较小，但如果左心室腔位于心尖，那么可以预期左心室功能能够耐受原发孔关闭后的容量负荷。