EBSTEINS ANOMALY: A RARE CARDIAC DISORDER UNVEILED BY SUDDEN ARRHYTHMIA IN A PREVIOUSLY STABLE ADULT

Ebsteins anomaly (EA) is a rare congenital heart disease which occurs in approximately 1 in 200,000 live births and represents less than 1% of all cases of congenital heart disease(1). AE is a rare cardiopathy of the right ventricle that originates in an embryological peculiarity of the tricuspid valve responsible for over-apical implantation of the septal leaflet of the tricuspid valve (2). We report the case of a 47-year-old patient with asymptomatic EA, admitted with junctional tachycardiaat 200bpm and haemodynamic instability. Due to this instability, we administred a synchronous external electric shock at 150 joules after sedation. The patient immediately recovered regular sinus rhythm and achieved haemodynamic stability. Echocardiographic evaluation supported the of EA, showing apical displacement of the tricuspid valve and a mitro-tricuspid mismatch of 11.6 mm/m2, The septal leaflet exhibited reduced mobility, attached to the interventricular septum, and the right atrium was dilated due to right ventricular atrialization. The clinical presentation of EA varies from neonatal respiratory distress to right heart failure and rhythm disorders in adolescents and adults. Some cases exhibit exceptionally long survival. Management of EA depends on its anatomical form and clinical presentation (3). Patients with minor tricuspid valve displacement are often asymptomatic and do not require specific treatment, Surgery is indicated when the patient is symptomatic or when arrhythmias or echocardiographic changesare detected (4).This clinical presentation highlights the rarity of such cases in our practice.