Electrophysiologic characteristics of Ebstein's anomaly of the tricuspid valve.

Electrophysiologic characteristics of five patients with Ebstein's anomaly of the tricuspid valve were defined with studies using luminal intracardiac electrode catheters. The diagnosis was made in each case from clinical data and confirmed at cardiac catheterization by the presence of an atrialized right ventricular chamber with atrial mechanical activity and ventricular electrical activity. In three cases intra-right atrial conduction was prolonged (P-A intervals of 50, 50, and 65 msec), a finding which reflected the presence of a characteristically large right atrium. The bundle of His electrogram was recorded in its usual anatomical location. Atrioventricular nodal conduction was prolonged in only one case. Intra-His delay was observed in two cases (bundle of His duration of 30 and 30 msec). Infranodal conduction was prolonged in four cases with H-V intervals of 60, 65, 65, and 80 msec. The anatomical abnormalities were least severe in the only patient with a normal H-V interval (50 msec). The prolonged H-V interval was thought to result from stretching of the conduction system over the atrialized right ventricle (ARV). The late depolarization during the splintered R' of the electrocardiogram found during intracardiac mapping of the ARV in three patients confirms the theory that the ARV produces the "second QRS" typically seen in this anomaly. The ARV was particularly irritable, and ventricular fibrillation was produced in two patients during catheter manipulation in this area. In one case the ARV had a shorter refractory period than the body of the right ventricle. Re-entrant supraventricular tachycardia was induced in the only patient with Wolff-Parkinson-White syndrome. In addition to the previously recognized electrophysiologic features reconfirmed here, patients with Ebstein's anomaly of the tricuspid valve usually have: normal position of the bundle of His, prolonged intra- right atrial conduction, prolonged infranodal conduction, and irritable ARV with delayed activation.