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纠正右肺至下腔静脉的异常静脉回流(弯刀畸形)。

CORRECTION OF ANOMALOUS VENOUS RETURN FROM THE RIGHT LUNG TO THE INFERIOR VENA CAVA (SCIMITAR DEFORMITY).

作者信息

Oglietti Jorge, Frazier O Howard, Reul George J., Cooley Denton A.

机构信息

Division of Surgery of the Texas Heart Institute, St. Luke's Episcopal and Texas Children's Hospital, Houston, Texas.

出版信息

Cardiovasc Dis. 1976;3(2):210-219.

Abstract

Anomalous pulmonary venous drainage to the inferior vena cava is a rare congenital cardiac defect. Oxygenated blood from the right lung enters the right atrium resulting in a left-to-right shunt. Because the radiographic shadow of the anomalous vein resembles a curved saber, this defect has been called the "scimitar" deformity. From 1958 through June 30, 1975, 11 patients underwent surgical correction of this anomaly at our institution with 10 survivors. Diagnosis was made by routine roentgenography of the chest in all but one patient. Eight patients had total correction which consisted of implanting the anomalous vein into the right atrium, opening the interatrial septum and applying a patch graft as a baffle to direct pulmonary venous blood into the left atrium. Five patients had associated cardiac defects which were also repaired. Three patients underwent pneumonectomy and all survived. The only operative death occurred in a 5-year-old female with an atrial septal defect and endocardial cushion defect. We believe the existence of a large left-to-right shunt justifies surgical intervention. The prognosis appears to depend upon the presence of other cardiac or pulmonary anomalies.

摘要

肺静脉异常引流至下腔静脉是一种罕见的先天性心脏缺陷。来自右肺的含氧血液进入右心房,导致左向右分流。由于异常静脉的X线影像类似弯刀,这种缺陷被称为“弯刀”畸形。从1958年至1975年6月30日,我们机构有11例患者接受了该畸形的手术矫正,其中10例存活。除1例患者外,所有患者均通过胸部常规X线检查确诊。8例患者接受了完全矫正,包括将异常静脉植入右心房、打开房间隔并应用补片作为挡板,将肺静脉血引流入左心房。5例患者伴有其他心脏缺陷,也进行了修复。3例患者接受了肺切除术,均存活。唯一的手术死亡发生在一名患有房间隔缺损和心内膜垫缺损的5岁女性患者身上。我们认为存在大量左向右分流是进行手术干预的合理依据。预后似乎取决于是否存在其他心脏或肺部异常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2909/287598/f1df1a72cba4/cardiodis00022-0099-a.jpg

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