Why the study?
Unilateral pulmonary vein atresia is rarely diagnosed in adulthood and can indirectly manifest as interstitial lung disease.
Highlights a rare adult presentation of unilateral pulmonary vein atresia mimicking interstitial lung disease, with documented radiographic changes over 12 years.
Consider unilateral PVA in adults with dyspnea, hemoptysis, or ILD-like changes; leaves open optimal diagnostic pathways for this rare entity.
Unilateral pulmonary vein atresia (PVA) is a rare congenital cardiovascular anomaly occurring after the common pulmonary vein fails to incorporate into the left atrium. It is most commonly diagnosed in childhood, and diagnosis after reaching adulthood is extremely rare. Dyspnea on exertion and hemoptysis are common clinical features in adult PVA patients, whereas lung parenchymal abnormalities are indirect signs of PVA, which can manifest as interstitial lung disease. Herein, we present the case of a 62-year-old female diagnosed with unilateral PVA presenting as unilateral interstitial lung disease and report the changes in her chest radiographs over 12 years.
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Sim et al. (2021) studied this question.
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