Mediastinal and hilar soft tissue mass‐like lesions in congenital unilateral pulmonary vein atresia: A retrospective review of seven pediatric patients

Abstract

Background: Congenital unilateral pulmonary vein atresia (CUPVA) is known to lead to the formation of an abnormal confluent mediastinal and hilar soft tissue mass, thoracic hypoplasia, and interlobular septal thickening on the affected side. The purpose of the present study is to investigate the frequency and severity of mediastinal soft tissue mass-like lesions and examine other abnormal findings associated with CUPVA. Methods: We retrospectively reviewed seven children with CUPVA who underwent contrast-enhanced CT scans and measured the soft tissue mass volume in the bilateral mediastinum (affected and normal side). The location of abnormal soft tissue was divided into three anatomical sections (paratracheal, peribronchial, and the dorsal aspect of the left atrium). The relationships among soft tissue volume and anatomical section were statistically evaluated. Also, the presence of thoracic hypoplasia, small ipsilateral pulmonary arteries, interlobular septal thickening, and ground-glass opacities were investigated. Results: In all cases, CT scans confirmed the presence of confluent soft tissue mass-like lesions in the affected mediastinum. The soft tissue volume on the affected side was 5.5-fold greater than the volume on the normal side (average: 18.0 cm 3 and 4.25 cm 3 respectively, P < 0.01). Thoracic hypoplasia and interlobular septal thickening were found in all patients. Small pulmonary arteries and ground-glass opacities were present in six of the seven patients. Conclusion: Abnormal mediastinal and hilar soft tissue is commonly found in patients with CUPVA. So, if we encounter the mediastinal soft tissue mass in patients with CUPVA, no further test will be indicated.

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

“…CUPVA is an extremely rare malformation with nearly 50 cases reported till 2018 [ 3 ] and just more than 12 case then after [ 4 , 11 – 15 ]. While congenital heart diseases may be found in up to 50% of patients, isolated CUPVA as in our patient is even more rare [ 3 ].…”

“…The increased risk of infection has various factors related to a hypoplastic lung, including impaired mucocilliary clearance, impaired local immunity, and impaired venous and lymphatic drainage. The typical radiographic features of CUPVA include a small ipsilateral hemithorax with reticular opacities and septal lines, pleural thickening or effusion, ipsilateral mediastinal shift, a small ipsilateral pulmonary artery, and absence of pulmonary venous drainage into the left atrium and ipsilateral mediastinal mass [ 1 – 11 ]. Reticular opacities in the smaller lung corresponds to thickened interlobular septae likely from chronic changes of venous hypertension and impaired lymphatic drainage while the decreased lung volume may be related to preferential arterial perfusion of the normal lung, impaired growth in the ipsilateral pulmonary artery, and chronic parenchymal scarring and changes [ 1 , 4 ].…”

“…Failure of incorporation of the pulmonary veins into the left atrium results in persistence of connections between the pulmonary circulation and the systemic veins that result in the presence of mediastinal collateral vessels. These collaterals may appear as a mediastinal mass on chest CT [ 9 – 11 ].…”

Unilateral pulmonary vein atresia: Literature overview and case report

“…The consolidation of the parenchyma may be attributed to the production of replacement tissue by the pulmonary veins. The noninvasive diagnosis of unilateral pulmonary vein atresia can be performed by CT angiography, cardiac magnetic resonance imaging (MRI), or bronchoscopy [ 10 , 11 ]. Similar to cardiac MRI, chest CT can provide precise morphological information regarding the structures of the heart and extracardiac vasculature [ 9 , 12 , 13 ].…”

Machine Learning-Based MRI LAVA Dynamic Enhanced Scanning for the Diagnosis of Hilar Lesions

“…Hilar lesions are a widespread disease, especially cholangiocarcinoma [ 13 ]. At present, dynamic enhanced sequence MRI LAVA is the best imaging method for diagnosing and differential hilar lesions [ 14 ]. By obtaining dynamic enhanced multiphase images, more accurate information about blood perfusion of hilar lesions can be provided.…”