Recurrent Transient Ischemic Attacks in a 48-Year-Old Patient With an Undiagnosed Ebstein's Anomaly: A Case Report

Abstract

Ebstein ’ s anomaly is a rare congenital malformation of the heart characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Its presentation depends on the severity of the anomaly. Mild forms are often undiagnosed until adulthood due to its benign course. Importantly, interatrial communications are present in 80% of the patients with EA through which a paradoxical embolism may occur whenever a right-to-left shunt exists. The authors describe a case of a 47-year-old white man that presented with acute recurrent transitory ischemic brain attacks and progressive exertional dyspnea. A brain CT scan revealed infarcts in multiple vascular territories. Echocardiographic studies revealed an Ebstein ’ s anomaly with a right-to-left shunt through an interatrial communication and severe right heart failure. Doppler study of the cervical and extremities ’ vessels was negative for a possible thrombotic source. A diagnosis of transient ischemic attacks presumably secondary to paradoxical embolism with unknown source was established. The patient was successfully submitted to heart surgery and improvement of symptoms and heart function was observed. At follow-up he remains well and without further neurological events. doi:10.4021/jmc439w