Diagnosis of ebstein anomaly with atrial septal defect and persistent left superior vena cava using cardiac magnetic resonance imaging

초록

A 26-year-old man was admitted to the hospital with acute onset of palpitation. His electrocardiogram revealed atrial fibrillation. Transthoracic echocardiography showed a huge coronary sinus and apical displacement of the tricuspid septal leaflets (Figure 1A-B and Movie 1). We suspected the Ebstein anomaly and tried to find atrial septal defect (ASD), which is often accompanied by such anomalies. But we could not find this defect because he had a very poor acoustic window, and the heart structure was hidden by a huge coronary sinus. To identify persistent left superior vena cava (PLSVC) that may be causing huge coronary sinus, we performed contrast echocardiogram with agitated saline. After infusing saline via the left antecubital vein, the coronary sinus is filled with agitated saline before right atrial enhancement (Figure 1C and Movie 2). This process suggested the existence of PLSVC. For detailed confirmation, the patient underwent cardiac magnetic resonance (CMR) imaging. Cine images showed apical displacement of the septal leaflet from the insertion of the anterior leaflet of the mitral valve by 1.35 mm/m2 body surface area, consistent with Ebstein anomaly. In addition, 12-mm-sized secundum type ASD and presence of PLSVC were revealed (Figure 2 and Movie 3). © 2020 Korean Society of Echocardiography.