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[1例罕见的完全性肺静脉异位连接至右心房合并三房心病例]

[A rare case of total anomalous pulmonary venous connection to the right atrium confounding with cor triatrium].

作者信息

Fukazawa M, Orita H, Hirooka S, Gotoh S, Washio M

机构信息

Second Department of Surgery, Yamagata University School of Medicine, Japan.

出版信息

Kyobu Geka. 1994 Apr;47(4):295-8.

PMID:8152176
Abstract

Three-days-old newborn baby underwent ligation of the ductus arteriosus under the diagnosis of TAPVC and PDA with high flow heart failure. Her UCG showed the direct connection of common pulmonary venous chamber (CPVC) to the upper and medial portion of right atrium. CPVC positioned just above the left atrium but does not communicated with the left atrium. These findings characterized this anomaly as II A cor triatriatum classified by Lucus-Schumidt. At 5-month-old, corrective operation was performed. Both right and left venous chambers joined just before the connection to right atrium and drained to right atrium at the upper portion of atrial septum. CPVC and left atrium had independent wall. Accordingly, operative diagnosis was made as TAPVC (IIb). Intraatrial routing was performed using porcine pericardial patch. At 6 months after operation, patient is doing well without the sign of venous obstruction. This type of TAPVC is rare and may confound with cor triatriatum. Our case may suggest the pathological mechanism of cor triatriatum and that at least some of cor triatriatum may be identical with TAPVC.

摘要

一名三日龄新生儿在诊断为完全性肺静脉异位连接(TAPVC)和动脉导管未闭(PDA)并伴有高流量心力衰竭的情况下接受了动脉导管结扎术。她的超声心动图(UCG)显示共同肺静脉腔(CPVC)与右心房的上部和内侧部分直接相连。CPVC位于左心房上方,但与左心房不连通。这些发现将这种异常特征化为Lucus-Schumidt分类的II A型三房心。在五个月大时进行了矫正手术。左右静脉腔在连接到右心房之前汇合,并在房间隔上部引流至右心房。CPVC和左心房有独立的壁。因此,手术诊断为TAPVC(IIb型)。使用猪心包补片进行了心房内路径重建。术后六个月,患者情况良好,没有静脉阻塞的迹象。这种类型的TAPVC很罕见,可能与三房心混淆。我们的病例可能提示了三房心的病理机制,并且至少部分三房心可能与TAPVC相同。

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