Kawahito Tomohisa, Takano Shinji, Egawa Yoshiyasu, Iwamura Yoshinobu, Nakahara Yasuo, Nii Akira, Ohnishi Tatsuya, Miyagi Yuhichi, Terada Kazuya, Ohta Akira
Department of Cardiovascular Surgery, National Hospital Organization, Kagawa Children's Hospital, Zentsuji, Japan.
Kyobu Geka. 2013 Jul;66(7):545-50.
Pulmonary artery sling is frequently combined with tracheal stenosis, and occasionally combined with congenital heart defects. However, there are few reports of successfully treated cases that were combined with single ventricle. In this article, we report a successfully treated case of pulmonary artery sling combined with tracheal stenosis, single ventricle, pulmonary atresia, vascular ring, and bilateral superior vena cava. A male infant was referred to our hospital for central cyanosis, and was diagnosed with single ventricle (tricuspid stenosis, multiple ventricular septal defect, and hypoplastic right ventricle)with pulmonary atresia by echocardiogram. Tracheal stenosis was shown at cardiac catheterization. Pulmonary artery sling and tracheal diverticulum were diagnosed by computed tomography (CT) and magnetic resonance imaging(MRI)examination. Furthermore, the patient was complicated by vascular ring, which consisted of right aortic arch, an aberrant left subclavian artery, and patent ductus arteriosus, and this ductus arteriosus was connected to the left subclavian artery and pulmonary arterial trunk. After 6 months of medical treatment, including continuous infusion of prostaglandin, re-evaluation was performed by cardiac catheterization. We considered that bidirectional cavo-pulmonary shunt was appropriate for the patient since his pulmonary vasculature had matured well. An operation was performed under the use of cardio-pulmonary bypass. Release of vascular ring by division of the ductus, bilateral bidirectional cavo-pulmonary shunt, and a slide tracheoplasty for tracheal stenosis were performed simultaneously. His recovery was uneventful, and he is currently waiting to receive a Fontan-type operation.
肺动脉吊带常合并气管狭窄，偶尔合并先天性心脏缺陷。然而，合并单心室的成功治疗病例报道较少。在本文中，我们报告了一例成功治疗的肺动脉吊带合并气管狭窄、单心室、肺动脉闭锁、血管环和双侧上腔静脉的病例。一名男婴因中心性发绀转诊至我院，经超声心动图诊断为单心室（三尖瓣狭窄、多发室间隔缺损和右心室发育不良）合并肺动脉闭锁。心导管检查显示气管狭窄。通过计算机断层扫描（CT）和磁共振成像（MRI）检查诊断为肺动脉吊带和气管憩室。此外，患者还合并血管环，由右位主动脉弓、迷走左锁骨下动脉和动脉导管未闭组成，该动脉导管连接左锁骨下动脉和肺动脉主干。经过6个月的药物治疗，包括持续输注前列腺素，通过心导管检查进行了重新评估。鉴于患者的肺血管已发育良好，我们认为双向腔肺分流术适合该患者。在体外循环下进行了手术。同时进行了动脉导管切断以解除血管环、双侧双向腔肺分流术以及针对气管狭窄的滑动气管成形术。他恢复顺利，目前正在等待接受Fontan类手术。
