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.