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
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes