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Updated: May 13, 2025
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Three-Dimensional Echocardiographic Method for the Visualization and Assessment of Specific Parameters of the Pulmonary Veins
Published on: October 28, 2020
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Unilateral pulmonary vein atresia
1Respiratory Department, Children's Hospital Affiliated to Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Zhengzhou, China.
IJTLD Open
|April 14, 2025
Summary
Unilateral pulmonary vein atresia (UPVA) is a rare condition. This study found no significant clinical differences between Chinese patients and those reported globally, highlighting consistent disease characteristics.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Rare Diseases
Background:
- Unilateral pulmonary vein atresia (UPVA) is a rare congenital anomaly affecting pulmonary venous return.
- Understanding its clinical spectrum is crucial for diagnosis and management.
Purpose of the Study:
- To summarize the clinical characteristics of UPVA.
- To compare Chinese UPVA cases with the global published data.
Main Methods:
- Retrospective analysis of 6 Chinese pediatric UPVA cases.
- Systematic review of 79 previously reported UPVA cases worldwide.
- Comparison of demographic, clinical, imaging, treatment, and prognostic data.
Main Results:
- UPVA cases are sporadically distributed globally, with notable numbers in China and the US.
- Median age at diagnosis was 5.2 years; male-to-female ratio was 1:1.
- Common manifestations include recurrent pneumonia (79.2%), hemoptysis (48.1%), and exercise intolerance (35.1%).
- Comorbidities include congenital heart disease (34.1%) and pulmonary hypertension (20%).
- Overall mortality rate was 8.9%.
Conclusions:
- No statistically significant differences were found in clinical characteristics between Chinese UPVA patients and the global cohort.
- The findings support a consistent clinical profile of UPVA across different populations.
