2 PRESENTATION OF CASE
A 35 year-old lady was referred to adult congenital heart disease department due to recently presented dyspnea on exertion and systolic murmur. Transthoracic echocardiography indicated an enlarged RA with apical displacement of septal (1.6 cm/m2), posterior (1.5 cm/m2) and also anterior (1.2 cm/m2) tricuspid leaflets (figure 1). Severe right atrium enlargement and severe low gradient tricuspid regurgitation (TRG=26 mmhg) was also recorded. Interatrial septum was aneurysmal with large PFO. Left ventricular function was mildly reduced with hypertrabeculation and apical non-compaction. Chest X ray showed increased cardiothoracic ratio with enlarged RA size (figure 2). Electrocardiogram showed first degree AV block, Q in leads III and aVF and narrow QRS (figure 3). Cardiac magnetic resonance (CMR) was done to evaluate cardiac function, anatomy as well as fibrosis. It was demonstrated an extraordinary case of Ebstein anomaly with apical displacement of all anterior (35 mm/m2 BSA), septal (18 mm/m2 BSA) and posterior (20 mm/m2 BSA) tricuspid valve leaflets (figure 4). Anterior trictspid valve leaflet was short and had some fenestration with severe TR. CMR also mentioned a prominent ridge in RA at the site of AV groove, which protruded to RV cavity with attachment of anterior TV leaflet to it.