Presentation
Dyspnea and recurrent pneumonia.
Patient Data
The heart is shifted to the right side. There is a large radio-opaque structure lateral to the right heart border extending below the diaphragmatic copula. Note the right upper zonal opacity.
Enlarged heart with a defect in the interventricular septum. Dilated main pulmonary trunk and left the main branch with a relatively reduced size of the right one. A hypoplastic right lung is noted with posterior trans-mediastinal herniation. There is an abnormal vein draining the right lung to the supra-hepatic IVC. Note the sequestrated portion of the right lung supplied by a branch from the abdominal aorta.
Abnormal vein (scimitar-shaped) draining into the supra-hepatic IVC. Note the abnormal branch originating from the abdominal aorta directed towards the right lung.
Abnormal vein (red arrow) draining into the supra-hepatic IVC (yellow arrow).
Case Discussion
Scimitar syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right lung into the inferior vena cava. The abnormal vein may drain into the inferior vena cava itself, the portal vein, or the hepatic veins. It is associated with hypoplasia of the right lung, pulmonary sequestration, and dextro-position of the heart.
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