Clinical characteristics of pulmonary vein stenosis in children
10.3760/cma.j.cn101070-20220819-00990
- VernacularTitle:儿童肺静脉狭窄临床特征分析
- Author:
Lanqin CHEN
1
;
Yao YAO
;
Ju YIN
;
Xiuyun LIU
;
Qiang QIN
;
Chenghao CHEN
;
Baoping XU
;
Kunling SHEN
Author Information
1. 国家儿童医学中心，国家呼吸系统疾病临床研究中心，首都医科大学附属北京儿童医院呼吸科，中国医学科学院儿童危重感染诊治创新单元，北京 100045
- Keywords:
Pulmonary vein stenosis;
Anomalous pulmonary venous connection;
Congenital heart disease;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2023;38(4):296-299
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.
