Circulation, Vol 52, 987-995, Copyright © 1975 by American Heart Association
JA Kastor, BN Goldreyer, ME Josephson, JK Perloff, DL Scharf, JH Manchester, JC Shelburne and JW Hirshfeld Jr
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.
ARTICLES
Electrophysiologic characteristics of Ebstein's anomaly of the tricuspid valve
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