An Adult-Onset Patient With Scimitar Syndrome: An Anatomical and Functional Evaluation by Cardiac Magnetic Resonance Imaging

Abstract

Scimitar syndrome is a rare congenital heart disease that presents with a partial anomalous venous connection and a hypoplastic right lung. It has been reported in 0.5% to 1% of all congenital heart diseases. A partial anomalous pulmonary venous structure (scimitar syndrome) draining into the inferior vena cava was detected in a 37-year-old female patient, who presented with mild dyspnea and chest pain. The anomalous pulmonary venous drainage into the inferior vena cava (scimitar syndrome) was diagnosed by cardiac magnetic resonance imaging (CMR) and 3D contrast-enhanced magnetic resonance angiography, which demonstrated the anomalous pulmonary venous connection with a pulmonary-to-systemic flow ratio noninvasively. The patient was referred for surgery without additional interventional imaging. She had a good postoperative course. We herein present 3D magnetic resonance angiography and phase-contrast CMR findings in an adult-onset patient with scimitar syndrome.