Y. Wang, Y. Tang, L. Sun, X. Zhang, Y. Liu, S. Xu, Y. Su, L. Zhang, X. Tang, H. Yang, Yuelin Shen
Henan Children’s Hospital, Zhengzhou Children’s Hospital and Zhengzhou University. Capital Medical University and National Center for Children’s Health. Children’s Hospital of Xinjang Uygur Autonomous Region, Xinjiang Hospital of Beijing Children’s Hospital and Seventh People’s Hospital of Xinjiang Uygur Autonomous Region.
China
International Union Against Tuberculosis and Lung Disease Open
IJTLD Open 2025; 2: 224-229
DOI: 10.5588/ijtldopen.24.0631
Abstract
Objective: This study aims to summarise the clinical characteristics of unilateral pulmonary vein atresia (UPVA) and compare the differences between Chinese cases and all published cases worldwide.
Methods: We retrospectively enrolled 6 Chinese children with UPVA from January 2014 to January 2024 at a single centre. We reviewed their demographic data, clinical symptoms, laboratory tests, imaging examinations, treatment and prognosis to describe their clinical features. Additionally, the remaining 79 confirmed patients with UPVA, as described in 52 references, were also summarised.
Results: UPVA is sporadically distributed worldwide, with the highest number of reported cases in China (27/85) and the United States (20/85). In the overall cohort (85 cases), the patient median age at diagnosis was 5.2 years. The male-to-female ratio was 1:1. Right-sided UPVA was slightly more common, with a right-to-left ratio of 1.4:1. The most frequently reported clinical manifestations were recurrent pneumonia (79.2%), followed by recurrent haemoptysis (48.1%) and exercise intolerance (35.1%). Additionally, 10.4% of patients were asymptomatic. Congenital heart disease was observed in 34.1% of cases, and 20% of patients had comorbid pulmonary hypertension. The overall mortality rate was 8.9%.
Conclusion: There were no statistically significant differences in the clinical characteristics of UPVA between Chinese patients and all published cases worldwide.
Category
Absence or Atresia of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Symptoms and Quality of Life Associated with Pulmonary Venous Obstruction
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2025
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients). Literature Review and Analysis.
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes