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中国循证儿科杂志

中国循证儿科杂志 ›› 2025, Vol. 20 ›› Issue (1): 57-61.DOI: 10.3969/j.issn.1673-5501.2025.01.010

• 论著 • 上一篇    下一篇

右肺动脉缺如37例超声心动图特征的病例系列报告

刘徐妹1,2,李健茹1,梁小碧1,陆叶1,马力1,王莉1   

  1. 1 广州医科大学附属妇女儿童医疗中心心脏中心 广州，510623；2广州市妇女儿童医疗中心柳州医院超声科 柳州，545006


  • 收稿日期:2024-08-26 修回日期:2025-01-16 出版日期:2025-02-25 发布日期:2025-02-25
  • 通讯作者: 王莉

Echocardiographic features of 37 cases with absent right pulmonary artery: A case series report

LIU Xumei1,2, LI Jianru1, LIANG Xiaobi1, LU Ye1, MA Li1, WANG Li1   

  1. 1 Heart Center, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou 510623, China; 2 Ultrasound Department, Liuzhou Hospital, Guangzhou Women and Children's Medical Center,  Liuzhou 545006, China
  • Received:2024-08-26 Revised:2025-01-16 Online:2025-02-25 Published:2025-02-25
  • Contact: WANG Li

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摘要： 背景：经胸超声心动图（TTE）是右肺动脉缺如（UARPA）术前和术后随访评估的重要检查方法：：，关于其超声心动图特征、手术前后肺动脉压力变化的报道较为少见。 目的：探讨和总结UARPA患儿手术前后超声心动图特征。 设计：病例系列报告。 方法：：回顾性收集广州医科大学附属妇女儿童医疗中心（我院）2018年1月至2024年1月确诊为UARPA的连续病例，于我院病历系统中采集患儿性别、年龄、体重，临床表现、手术前后辅助检查结果：、治疗、随访时间及随访结果：，并进行总结。 主要结局指标：手术前后超声心动图特征。 结果：37例UARPA患儿进入本文分析，男18例，女19例，中位年龄7个月（1日龄至6岁9个月），25例（67.6%）年龄＜1岁，体重6.4（2.7~22.1）kg。主要临床表现为气促（21.6%）、反复呼吸道感染(18.9％)、反复咯血和发绀(各16.2％)。27例（73.0%）合并其他心内畸形，其中房间隔缺损或卵圆孔未闭23例（62.2%），动脉导管未闭12例（32.4%），迷走右锁骨下动脉1例（2.7%）。合并肺动脉高压19例（51.4%）。超声心动图典型特征为主肺动脉远端未见右肺动脉发出，主肺动脉直接延续为左肺动脉。右肺主要通过主动脉及弓部分支血管来源的动脉导管未闭或侧枝血管供血。 27例患儿行右肺动脉重建手术，中位手术年龄5月龄（11 d至4岁9月龄），术后随访时间8（1~52）个月。末次随访肺动脉高压患儿7.4%(2/27)，右肺动脉吻合口狭窄14.8%（4/27），重建的右肺动脉血流速度（2.1±0.7）m·s-1。 结论：UARPA主要合并畸形为房间隔缺损或卵圆孔未闭、动脉导管未闭，容易继发肺动脉高压，行肺动脉重建术后效果较好，TTE对于UARPA的诊断和术后评估有重要价值。

关键词: 右肺动脉缺如, 超声心动图, 儿童

Abstract: Background： Objective：Transthoracic echocardiography (TTE) is an important examination method for preoperative and postoperative follow-up assessment of absent right pulmonary artery (UARPA). Reports on its echocardiographic features and changes in pulmonary artery pressure before and after surgery are relatively rare. Design：Case series reports. Methods：A retrospective collection of consecutive cases diagnosed with UARPA at Guangzhou Women and Children's Medical Center, Guangzhou Medical University was conducted from January 2018 to January 2024. Data on patients' gender, age, weight, clinical manifestations, preoperative and postoperative auxiliary examinationResults：, treatment, follow-up duration, and follow-up outcomes were extracted from the hospital's medical record system and summarized. Main outcome measures：Echocardiographic features before and after surgery. Results：A total of 37 pediatric patients with UARPA were included in this study, consisting of 18 males and 19 females. The median age was 7 months (ranging from 1 day to 6 years and 9 months), with 25 cases (67.6%) under the age of 1 year. The median body weight was 6.4 kg (range: 2.7-22.1 kg). The main clinical manifestations included tachypnea (21.6%), recurrent respiratory infections (18.9%), and recurrent hemoptysis and cyanosis (16.2% each). Among the 37 cases, 27 (73.0%) had associated congenital heart defects, with the most common being atrial septal defect (ASD) or patent foramen ovale (PFO) in 23 cases (62.2%) and patent ductus arteriosus (PDA) in 12 cases (32.4%). Additionally, one patient (2.7%) had an aberrant right subclavian artery. Pulmonary hypertension was observed in 19 patients (51.4%). Echocardiographic examination revealed that the right pulmonary artery was absent at the distal main pulmonary artery, with the main pulmonary artery continuing directly into the left pulmonary artery. The right lung received blood supply through PDA or collateral vessels originating from the aorta and its arch branches. Among the 37 patients, 27 (73.0%) underwent right pulmonary artery reconstruction surgery, with a median surgical age of 5 months (range: 11 days to 4 years and 9 months). The median postoperative follow-up period was 8 months (range: 1-52 months). At the last follow-up, only two patient (7.4%) had residual pulmonary hypertension, and four patient (14.8%) developed anastomotic site stenosis of the reconstructed right pulmonary artery. The mean blood flow velocity in the reconstructed right pulmonary artery was 2.1 ± 0.7 m·s-1. Conclusion：UARPA is frequently associated with atrial septal defect (ASD), patent foramen ovale (PFO) or patent ductus arteriosus (PDA) and is prone to secondary pulmonary hypertension. Pulmonary artery reconstruction surgery demonstrates favorable outcomes, with a low incidence of postoperative complications. Transthoracic echocardiography (TTE) plays a crucial role in the diagnosis and postoperative assessment of UARPA.

Key words: Unilateral absence of right pulmonary artery, Echocardiography, Children