Transcatheter Management of Atypical Scimitar Syndromes With Dual Venous Drainage

The scimitar syndrome is a rare congenital condition including a partial anomalous pulmonary venous return from the right lung into the inferior vena cava (IVC) which leads to a left-to-right shunt. Atypical variants with a dual drainage to both the IVC and the left atrium may be amenable to transcatheter occlusion. We report 3 patients diagnosed with scimitar syndrome with this dual drainage. Explorations confirmed the connection of the scimitar vein (SV) into both the IVC and the left atrium, with a significant left-to-right shunt and a right ventricular dilation. Pulmonary pressures were normal or mildly elevated. A compliant balloon occlusion test was performed at the IVC connection of the SV. All the patients underwent successfully a transcatheter occlusion of SV at the IVC junction using occlusion devices allowing proximal re-rerouting to the left atrium through the fistula. Aberrant systemic artery arising from the abdominal aorta was embolized in 2 cases during the same intervention.