Liu C Y, Chiu I S, Chen J J, Hung C R, Lien W P
National Taiwan University Hospital, Taipei, R.O.C.
J Formos Med Assoc. 1991 Jan;90(1):94-8.
Atrial isomerism is very rare in adolescence. Two cases of left atrial isomerism are reported here in 2 females, aged 21 and 19 years. They had presented with cyanosis and dyspnea since childhood. High kilovoltage filter films showed a bilateral morphologically left bronchus. Cardiac catheterization in Case 1 revealed normal pulmonary artery pressure, severe subvalvular pulmonic stenosis, a double outlet right ventricle, a significant oxygen step-up at the atrial level and moderate systemic oxygen desaturation; while Case 2 disclosed pulmonary hypertension and mild systemic oxygen desaturation. Both cases had the following anatomical features: ipsilateral connection of pulmonary veins to the bilateral morphological left atrium; interrupted inferior vena cave with azygos or hemiazygos continuation; total anomalous hepatic venous return to the right-sided atrium; complete atrioventricular canal. The diagnoses were confirmed in both cases at surgical correction.
心房异构在青少年中非常罕见。本文报告了2例左心房异构的女性病例，年龄分别为21岁和19岁。她们自童年起就出现了紫绀和呼吸困难。高千伏滤线片显示双侧形态学上的左支气管。病例1的心导管检查显示肺动脉压正常、重度瓣膜下肺动脉狭窄、右心室双出口、心房水平明显的氧分压升高以及中度体循环氧饱和度降低；而病例2则显示有肺动脉高压和轻度体循环氧饱和度降低。两例均具有以下解剖学特征：肺静脉同侧连接至双侧形态学上的左心房；下腔静脉中断，奇静脉或半奇静脉延续；肝静脉完全异常回流至右侧心房；完全性房室通道。两例在手术矫正时均确诊。
