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Unilateral pulmonary vein atresia
AJR. American Journal of Roentgenology
|October 1, 1980
Summary
Unilateral pulmonary vein atresia in children presents with hemoptysis and recurrent infections. Definitive diagnosis involves imaging, with treatment typically requiring surgical removal of the affected lung.
Area of Science:
- Pediatric Pulmonology
- Cardiovascular Imaging
- Congenital Abnormalities
Background:
- Unilateral pulmonary vein atresia is a rare congenital condition.
- It is often associated with focal congenital pulmonary vein stenosis.
Observation:
- Three pediatric cases presented with hemoptysis, recurrent pulmonary infections, and a small or normal-sized lung with reticular markings.
- Clinical presentation suggests a strong suspicion for the diagnosis.
Findings:
- Radionuclide perfusion studies and pulmonary angiography confirm decreased blood flow to the affected lung.
- Pulmonary arteriography is definitive, revealing a small pulmonary artery, pruned branches, contrast stasis, and non-visualized pulmonary veins.
Implications:
- Early suspicion and diagnostic confirmation are crucial for appropriate management.
- Surgical resection of the affected lung or lobe is the standard treatment.
- Understanding this condition aids in diagnosing and managing rare pediatric lung diseases.