Transcatheter correction of the scimitar variant with dual pulmonary venous drainage-an international multicentre series.

BACKGROUND

Scimitar syndrome is an uncommon congenital malformation of pulmonary venous drainage to the junction of the right atrium and inferior caval vein. Treatment is usually surgical, depending on the morphological variant. When there is dual drainage of the anomalous veins to the inferior caval vein and the left atrium, a transcatheter procedure may represent an alternative to surgery.

METHODS

We report a series of four patients with the scimitar variant with dual pulmonary venous drainage treated with a transcatheter approach.

RESULT: All four patients (three children, one adult) had dual drainage of right pulmonary veins into the inferior caval vein and to the left atrium via a connecting vein. All patients underwent a successful catheter occlusion of the anomalous connection to the systemic vein without complication. Vascular plug devices were used in two patients: a left atrial appendage closure device in one patient and a ventricular septal defect closure device in one patient. All the procedures resulted in complete occlusion of the anomalous venous drainage to the inferior caval vein and unobstructed drainage to the left atrium.