RT Book, Section A1 Kay, W. Aaron A1 Roble, Sharon A1 Zaidi, Ali N. A2 Daniels, Curt J. A2 Zaidi, Ali N. SR Print(0) ID 1128634127 T1 THE ADULT WITH EBSTEIN's ANOMALY OF THE TRICUSPID VALVE T2 Color Atlas and Synopsis of Adult Congenital Heart Disease YR 2016 FD 2016 PB McGraw-Hill Education PP New York, NY SN 9780071749435 LK accesscardiology.mhmedical.com/content.aspx?aid=1128634127 RD 2024/03/29 AB A 32-year-old woman presented for evaluation of decreasing exercise tolerance and a loud heart murmur. On physical examination, she had a fixed split second heart sound with a normal pulmonary component. She had a holosystolic murmur at the mid-left sternal border that did not radiate and increased with inspiration. An electrocardiogram (ECG) and echocardiogram were performed. ECG demonstrated right atrial enlargement and a right bundle branch block. Echocardiogram showed apical displacement of the septal and posterior leaflets of her tricuspid valve resulting in severe tricuspid regurgitation. Her clinical findings were consistent with Ebstein's anomaly. Further testing included a functional vo2 exercise stress test. With exercise, she was found to have significant oxygen desaturation to the mid-80s on room air. A subsequent transesophageal echocardiogram showed a patent foramen ovale (PFO) with bidirectional shunting, severe tricuspid regurgitation, and an apically displaced septal leaflet of the tricuspid valve consistent with Ebstein's anomaly. Because of her significant hypoxia with exercise, she underwent a catheter-based closure of her PFO. A repeat exercise study following PFO closure showed normal oxygen saturations with exercise and improved exercise capacity. She was followed with serial imaging that continued to demonstrate the severe tricuspid regurgitation and gradual worsening in her functional capacity. She developed atrial flutter with rapid ventricular rate for which she needed to be cardioverted. She was managed with antiarrhythmic medications and has had no further arrhythmia recurrences. She finally underwent surgical management in the form of a tricuspid valve replacement with a bioprosthetic valve and plication of the atrialized ventricle. The patient had an uneventful postoperative recovery with significant improvement in her functional capacity.