Unilateral pulmonary vein atresia presenting with recurrent hemoptysis and bronchial varices in an Ethiopian adolescent: a case report

Abate Yeshidinber Weldetsadik, Abdi Kebede, Binyam Gebremedhin Godu, Maru Gama
Saint Paul’s Hospital Millennium Medical College.
Ethiopia

Journal of Medical Case Reports
J Med Case Rep 2023; 17:
DOI: 10.1186/s13256-023-03956-4

Abstract
Background: Congenital unilateral pulmonary vein atresia is a rare anomaly resulting from failure of the pulmonary vein to incorporate in the left atrium. It is a very rare cause of recurrent respiratory infections and hemoptysis requiring a high index of suspicion for proper diagnosis and management in early childhood.
Case presentation: We report a 13-year old Anuac (Ethiopia, Region of Gambela) male adolescent with a delayed diagnosis of isolated atresia of the left pulmonary veins despite early childhood presentation with recurrent chest infections, hemoptysis and exercise intolerance. Contrast enhanced CT of thorax with reconstructed planes confirmed the diagnosis. He underwent pneumonectomy for severe and recurrent symptoms and did well on subsequent follow ups after 6 months of pneumonectomy.
Conclusion: Although a rare anomaly, congenital unilateral pulmonary vein atresia should be considered in the differential diagnosis of a child presenting with recurrent chest infections, exercise intolerance and hemoptysis to facilitate early appropriate diagnosis and treatment.

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: Filed (PHiled). Greater than 1-2 years since publication

Article Access Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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