66. Clinical profile of adult Ebstein’s anomaly

Abstract

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.