Lili Wang, Lin Zhong, Lina Chen, Hanmin Liu
West China Second University Hospital and Sichuan University.
China
BioMedical Central Pediatrics
BMC Pediatr 2025; 25:
DOI: 10.1186/s12887-025-06152-z
Abstract
Background: The absence of the pulmonary vein is a rare congenital cardiovascular anomaly. It is most commonly diagnosed in infants, and diagnosis in adolescence is rare. Here, we report the case of an 11-year-old girl with absence of the right pulmonary vein, initially misdiagnosed with interstitial lung disease.
Case presentation: This patient presented with recurrent haemoptysis, recurrent respiratory infection and decreased activity tolerance. The radiological findings included bronchiectasis, septal thickening, a small right lung with hypoplasia of the ipsilateral pulmonary artery and ipsilateral mediastinal shift. CTA revealed the absence of the right pulmonary vein. Surgery was recommended. However, the patient opted for conservative treatment and outpatient follow-up.
Conclusion: This report emphasizes the epidemiological factors, clinical features, and imaging findings of unilateral pulmonary vein atresia to prevent confusion and facilitate accurate diagnosis in similar cases. Unilateral PVA diagnosis should be considered when patients exhibit characteristic imaging features such as septal thickening, hypoplasia of the ipsilateral pulmonary artery and lung, compensatory dilation of the contralateral pulmonary artery and emphysema of the contralateral lung, coupled with clinical hallmarks including recurrent respiratory infection, haemoptysis, and decreased activity tolerance.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Symptoms and Findings Associated with Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
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