Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI

Abstract

We report an asymptomatic 8-year-old girl with anomalous pulmonary venous connection to the IVC and systemic arterial supply (scimitar syndrome). We present for the first time a description of gadolinium-enhanced 3D MR angiography that provided concurrent non-invasive complete anatomical (arterial and venous supply) and 'functional' (calculation of left-to-right shunt using phase-contrast-MRI performed in the ascending aorta, main pulmonary artery and anomalous pulmonary vein) diagnosis, avoiding the need for more traditional invasive techniques. As the shunt quantification was less than 2:1, conservative management was decided upon.