Congenital venolobar syndrome

Case contributed by Dr Rachael O'Rourke

Presentation

Known congenital heart disease with scimitar syndrome undergoing imaging work-up for preoperative planning

Patient Data

Age: Adult
Gender: Female

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

MRI

Pulmonary MRA demonstrates large right-sided scimitar vein draining to the distended IVC with right atrial and ventricular dilation, due to a long standing left to right shunt. A small systemic vessel arises from the aorta on the right and supplies the posterobasal segment of the right lower lobe, with a difference in perfusion seen within this pulmonary segment as a result. This represents a right lower lobe sequestration.

Incidental breast prostheses.

Case Discussion

Congenital venolobar syndrome in a young female patient, with large scimitar vein and right lower lobe pulmonary sequestration. Identification of the systemic vessel arising from the aorta is important for surgical planning, enabling the surgeon to avoid injury to the vessel and decrease perioperative risk.

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