Department of Pulmonology.
Department of Neonatology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang Province, China.
Medicine (Baltimore). 2021 Jan 22;100(3):e24021. doi: 10.1097/MD.0000000000024021.
Pulmonary artery sling (PAS) is a rare congenital anomaly. Associated airway anomalies and/or those of the cardiovascular system are present in about half the patients. Situs inversus is a rare disease in which organs of the chest and/or abdomen are arranged in a mirror image reversal of their normal position. Herein, we report a rare case of pulmonary artery sling and situs inversus incompletus, which has not yet been reported.
A 10-year-old girl was admitted because of heart murmur for more than 9 years. On physical examination, the second heart sound was prominent, and a grade 2/6 systolic murmur was heard at the left mid-sternal border. Echocardiography revealed PAS and atrial septal defect (8.6 mm). A chest computer tomography angiograph demonstrated that she had lung inversus, right aortic arch, and right lung hypoplasia in addition to PAS, with a normal positioning of the heart. The PAS intersected and twisted across the bronchus, which was obviously narrowed. The PAS was type II B, since the carina was at the T6 level without a separate right upper lobe bronchus.
Her final diagnosis was that of PAS, tracheal stenosis, situs inversus incompletus, right lung hypoplasia, right aortic arch, ASD and PDA.
She underwent one-stage total correction for her initial cardiovascular defects through median sternotomy under cardiopulmonary bypass support.
She had an uneventful recovery and completely healthy following the procedure.
A thorough examination before PAS surgery was essential in discovering and carefully evaluating complicated heart and lung anomalies.
肺动脉吊带（PAS）是一种罕见的先天性畸形。大约一半的患者存在相关的气道异常和/或心血管系统异常。内脏反位是一种罕见的疾病，其中胸部和/或腹部的器官以正常位置的镜像反转方式排列。在此，我们报告了一例罕见的肺动脉吊带和不完全性内脏反位病例，尚未有报道。
一名 10 岁女孩因心脏杂音超过 9 年而入院。体格检查时，第二心音明显，胸骨左缘中 2/6 级收缩期杂音。超声心动图显示 PAS 和房间隔缺损（8.6mm）。胸部计算机断层血管造影显示她除了 PAS 外，还存在肺反位、右主动脉弓和右肺发育不全，心脏位置正常。PAS 穿过并扭曲支气管，导致明显狭窄。PAS 为 IIB 型，因为隆嵴位于 T6 水平，没有单独的右上叶支气管。
PAS、气管狭窄、不完全性内脏反位、右肺发育不全、右主动脉弓、房间隔缺损和动脉导管未闭。
通过正中胸骨切开术在体外循环支持下进行了一期心血管缺陷的完全矫正。
她术后恢复顺利，完全健康。
PAS 手术前进行彻底检查对于发现和仔细评估复杂的心肺异常至关重要。
