# Unilateral pulmonary vein atresia

## TL;DR

This study summarizes the clinical features of unilateral pulmonary vein atresia and compares Chinese cases with global data, finding no significant differences.

## Contribution

The study provides a comprehensive comparison of UPVA cases in Chinese children with global data, highlighting similarities in clinical features.

## Key findings

- UPVA is most commonly reported in China and the United States, with a median age of diagnosis at 5.2 years.
- Recurrent pneumonia is the most common symptom, and right-sided UPVA is slightly more prevalent than left-sided.
- No significant differences in clinical features were found between Chinese and global UPVA cases.

## Abstract

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.

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.

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%.

There were no statistically significant differences in the clinical characteristics of UPVA between Chinese patients and all published cases worldwide.