66. Clinical profile of adult Ebstein’s anomaly

Adults often present with arrhythmias. In the presence of an interatrial communication, the risk of paradoxical embolization, brain abscess, and sudden death increases. 10% cases WPW syndrome, junctional rhythm with intermittent sinus pauses (20%) and ventricular trigeminy (10%). Overt cardiac failure was the initial presentation in (10% patients), whereas 20% patients presented with NYHA functional class II dyspnoea and easy fatiguability. Rheumatic fever was present coexisting with Ebsteins anomaly in (5%). Two patients presented with congenital rubella syndrome with patent ductus arteriosus with Ebsteins anomaly. 5% patients who presented with inferior wall myocardial infarction on routine echocardiographic evaluation had Ebsteins anomaly. Two patients had cyanosis. Associated defects in these patients included ASD (25%), ASD with VSD (20%), PDA (5%), RVOT aneurysm (5%), pericardial effusion (10%). All patients had low pressure tricuspid regurgitation (TR). Posterior leaflet displacement more than septal leaflet displacement was present in 20% patients. Anterior leaflet was not displaced in any of these patients. Celemajors index in most of the patients was in the range of (0.6–0.97). 3 patients had celemajor index of 1.5. Carpentier type I in 20% patients, in type II in 65% patients, type III in 10% patients, type IV in 5% patients. Cardiothoracic ratio ranged from 0.61 to 0.71 and only one patient had a ratio of 0.85. 10% cases WPW syndrome , junctional rhythm with intermittent sinus pauses (20%) and ventricular trigeminy (10%). Overt cardiac failure was the initial presentation in (10%patients), whereas 20% patients presented with NYHA functional class II dyspnoea and easy fatiguability. Rheumatic fever was present coexisting with Ebsteins anomaly in (5%). Two patients presented with congenital rubella syndrome with patent ductus arteriosus with Ebsteins anomaly. 5% patients who presented with inferior wall myocardial infarction on routine echocardiographic evaluation had Ebsteins anomaly. Two patients had cyanosis. Associated defects in these patients included ASD (25%), ASD with VSD (20%), PDA(5%), RVOT aneurysm (5%), pericardial effusion (10%). All patients had low pressure tricuspid regurgitation (TR). Posterior leaflet displacement more than septal leaflet displacement was present in 20% patients. Anterior leaflet was not displaced in any of these patients. Celemajors index in most of the patients was in the range of (0.6–0.97). 3 patients had celemajor index of 1.5. Carpentier type I in 20% patients, in type II in 65% patients, type III in 10% patients, type IV in 5% patients. Cardiothoracic ratio ranged from 0.61 to 0.71 and only one patient had a ratio of 0.85. Ebsteins anomaly in adults can have diverse presentations. Symptoms due to Electrophysiological events were more prevalent than due to hemodynamic disturbances