Scimitar Syndrome: An Uncommon Cause of Dyspnea in Adults
Image Modality: Magnetic Resonance Imaging Scan
A 43-year-old male patient with a medical history of hypertension presented to the cardiology clinic for evaluation of chest pain and dyspnea on exertion for several years. Physical exam revealed distant heart sounds. Electrocardiogram showed reversed R wave progression over precordial leads. Chest X-ray showed dextroposition of the heart with longitudinal density over the right hemithorax (A). Transthoracic echocardiography showed left ventricular ejection fraction of 55-60%, right ventricular (RV) dilation, and estimated RV systolic pressure of 45-50 mmHg consistent with moderate pulmonary hypertension. Myocardial stress imaging showed reversible defect in the inferolateral wall suggesting ischemia. Coronary angiography showed normal coronary arteries. Computed tomography angiography revealed dextroposition of the heart, hypoplastic right lung, right mediastinal shift on sagittal view (B), right superior pulmonary vein anomalous draining into the supra-hepatic portion of inferior vena cava on axial view (C, asterisk) and on three-dimensional rendered imaging (D, asterisk). The patient was diagnosed with Scimitar Syndrome, a rare congenital cardiopulmonary defect characterized by dextroposition of the heart, right lung hypoplasia, and anomalous right lung drainage into the systemic circulation. The presentation can vary from being asymptomatic to severe pulmonary hypertension and heart failure.1 The presence of limiting symptoms, pulmonary hypertension, and RV failure require correction.1 The patient was referred to cardiothoracic surgery for surgical repair.
- Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation 2010;122:1159-66.
Date: December 10, 2019
Clinical Topics: Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Acute Heart Failure, Pulmonary Hypertension, Interventions and Imaging, Interventions and Vascular Medicine, Angiography, Echocardiography/Ultrasound, Nuclear Imaging, Hypertension
Keywords: Diagnostic Imaging, Cardiac Imaging Techniques, Scimitar Syndrome, Coronary Angiography, Angiography, Heart Sounds, Pulmonary Veins, Blood Pressure, Vena Cava, Inferior, Coronary Vessels, Dilatation, Physical Exertion, Stroke Volume, X-Rays, Echocardiography, Electrocardiography, Chest Pain, Chest Pain, Hypertension, Pulmonary, Hypertension, Heart Failure, Dyspnea, Drainage