[与房间隔缺损、肺动脉狭窄及无左上腔静脉的完全性冠状静脉窦未闭相关的努南综合征：一例报告]

[Noonan syndrome associated with atrial septal defect, pulmonary stenosis, and completely unroofed coronary sinus without LSVC: a case report].

作者信息

Yoshihara K, Ozawa T, Sakuragawa H, Fujii T, Kawasaki M, Shiono N, Watanabe Y, Koyama N, Mastuura Y, Saji T, Takanashi Y

机构信息

Department of Thoracic Cardiovascular Surgery, Toho University School of Medicine, Tokyo, Japan.

出版信息

Kyobu Geka. 1999 Feb;52(2):134-7.

PMID:10036874

Abstract

An eight-year-old boy, Noonan syndrome associated with ASD and PS, was referred to our department for surgical repair. During operation, the coronary sinus ostium was not found. Farther more exploration revealed completely unroofed coronary sinus without LSVC. The large ASD (confluent with coronary sinus ASD) was closed with a ePTFE patch. The pulmonary valve was thickened moderately and each commissure was adhesive, but not dysplastic. PS was released with commissurotomy and subpulmonary muscle resection. The postoperative course was uneventful and the patient discharged at 14 postoperative day. At present, he has been followed at outpatient without PS and any sign and symptom of myocardial hypertrophy.

摘要

一名8岁男孩，患有与房间隔缺损（ASD）和肺动脉狭窄（PS）相关的努南综合征，因手术修复被转诊至我科。手术过程中，未发现冠状窦口。进一步探查发现冠状窦完全未闭且无左上腔静脉（LSVC）。巨大的房间隔缺损（与冠状窦型房间隔缺损相连）用膨体聚四氟乙烯（ePTFE）补片封闭。肺动脉瓣中度增厚，各瓣叶交界处粘连，但无发育异常。通过瓣交界切开术和肺动脉下肌切除术解除肺动脉狭窄。术后病程平稳，患者术后14天出院。目前，他在门诊接受随访，无肺动脉狭窄及任何心肌肥厚的体征和症状。