Presentation
Recurrent chronic cough
Patient Data
Small right hemithorax, ipsilateral diaphragmatic elevation, and ipsilateral mediastinum deviation likely indicate a hypoplastic right lung, while the contralateral lung is hyperinflated.
The right cardiac border is not visible. There is a curvilinear tubular structure with crescent morphology in the lower half of the right hemithorax, likely a scimitar vein.
A relatively dense rounded opacity corresponds with the superior portion of the scimitar vein which runs posterior to anterior in the right mid-thorax as seen on the subsequent CT scan.
There is evidence of right lung hypoplasia, indicated by a small volume of the right lung and decreased caliber of the pulmonary artery and bronchial tree. Additionally, there is dextroposition of the heart with hyperinflation of the contralateral lung.
The right inferior pulmonary vein is smaller in diameter than the left and drains into the left atrium. The right anomalous superior pulmonary vein has infracardiac venous drainage to the inferior vena cava. There was no visible systemic arterial supply to the hypoplastic lung.
There is a normal variant where the brachiocephalic artery and the left common carotid artery have a common origin (bovine arch). No evidence of pulmonary hypertension.
Case Discussion
The findings are suggestive of scimitar syndrome, which is also known as hypogenetic lung syndrome. This condition involves a partial anomalous pulmonary venous connection that affects the entire or a part of the right lung, as in this particular case. Notably, the scimitar vein tends to occur on the right side.
The clinical presentation of a patient with scimitar syndrome can vary depending on the size of the shunt and associated cardiopulmonary abnormalities. Infants may present with heart failure, whereas adults are often asymptomatic but may experience recurrent pulmonary infections linked to hypoplastic lung.
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