Unilateral Pulmonary Vein Atresia Initially Presenting as Interstitial Lung Disease in an Elderly Female: Serial Chest Radiograph Changes and Its Literature Review

Abstract

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.

Keywords: Computed Tomography, X-Ray; Interstitial Lung Disease; Pulmonary Vein.

Fig. 1. Radiologic findings of unilateral pulmonary vein atresia in a 62-year-old female, presenting as unilateral interstitial lung disease.

A. Axial image of the mediastinal window demonstrates a significant size discrepancy between the RPA and LPA. Apparent pleural thickening is noted along the right lung lateral margin. The hypertrophied hilar branch of the bronchial artery is noted (arrowheads). B, C. Lung window setting images show unilateral diffuse ground-glass opacities, reticulation, and traction bronchiectasis, with right lung volume loss. D. Serial chest radiographs taken in 2008, 2016, and 2020 (images from left to right). Initial chest radiograph (left) shows a small right hemithorax with right mediastinal shifting and diminished vessel size in the right lung. Right-mid and lower-zonal linear infiltration, reticular infiltration, and ground-glass opacities are visible. The follow-up chest radiograph (middle) demonstrates increased extent of linear, reticular infiltration, and ground-glass opacities with basal predominance in the right lung. The last follow-up chest radiograph (right) reveals progression of the reticular infiltrates, ground-glass opacities, and consolidations in the right lung. E. Posterior view of the volume-rendered reconstructed image of the chest CT, with removal of the descending aorta, shows the absence of right pulmonary venous drainage into the left atrium (arrow). The entirely smooth margin of the left atrium is noted (asterisk). LPA = left pulmonary artery, LPV = left pulmonary vein, RPA = right pulmonary artery