Ebstein’s malformation (EM) is characterized by dysplasia and displacement of the tricuspid inferior and septal leaflets from the true atrioventricular (AV) junction. Left ventricular hypertrabeculation/non-compaction (LVHT) including the ‘atrialized’ portion in EM has not been described. A 42-year-old man with a history of radiofrequency ablation of a Mahaim-like bundle suffered from chest pain. Coronary angiography was normal, but echocardiography

showed a septal tricuspid leaflet inserting 3.5-cm apically beyond the AV junction, deep recesses of the atrialized interventricular septum, and a heavily trabeculated left ventricle; these were confirmed by cardiac magnetic resonance imaging. Neurologically, hypoacusis, positive pyramidal signs, postural tremor and brisk tendon reflexes were identified.

The Journal of Heart Valve Disease 2006;15:719-720