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Ebstein’s Anomaly Unmasked by Accessory Pathway Ablation FLORIAN N. RIEDE, M.D., CHRISTIAN STICHERLING, M.D., STEFAN OSSWALD, M.D., and ¨ MICHAEL KUHNE, M.D. From the Department of Electrophysiology/Cardiology, University Hospital Basel, Basel, Switzerland

An 18-year-old male with a history of symptomatic tachycardia was referred for ablation. The ECG (Fig. 1, Panel A) showed preexcitation suggestive of a posteroseptal accessory pathway. Orthodromic AV-reentry tachycardia (ORT) with a wide right bundle branch block (RBBB) pattern was inducible (Fig. 1, Panel B). Mapping the right posteroseptal region in sinus rhythm showed a local activation time of –5 milliseconds, and activation was later at the left posterior septum. Mapping the inferior tricuspid annulus proved difficult despite the use of a deflectable sheath (Fig. 1, Panel J Cardiovasc Electrophysiol, Vol. 25, pp. 1261-1262, November 2014. S. Osswald reports educational grants from Medtronic, St. Jude Medical, Biotronik, Sorin, and Boston Scientific. Other authors: No disclosures. Address for correspondence: Michael K¨uhne, M.D., Department of Cardiology, University Hospital Basel, Petersgraben 4, 4031 Basel, Switzerland. Fax: +41-61-265-4598; E-mail: [email protected] doi: 10.1111/jce.12472

D). Echocardiography was performed and showed Ebstein’s anomaly with apical displacement of the septal tricuspid valve leaflet by 20 mm and moderate tricuspid valve regurgitation (Fig. 1, Panel E). No site with a clearly discernable atrial and early ventricular electrogram was found in that region. Ablation at a site on the inferior tricuspid annulus with a very early local activation time (–35 milliseconds, Fig. 1, Panel C, arrow) eliminated the accessory pathway. Note the marked local endocardial delay after ablation (Fig. 1, Panel C, arrowhead) due to preexisting RBBB and the ventricular location of the successful ablation site with only a very small atrial electrogram reflecting the difficulty of finding a good annular electrogram during mapping. Ebstein’s anomaly is a rare congenital disease associated with accessory pathways and RBBB. The RBBB may be masked by preexcitation at baseline, but the abnormal activation of the right ventricle leading to the wide RBBB pattern may be unmasked during ORT, as demonstrated in our patient (Fig. 1, Panel B), and this can be a clue to the diagnosis.

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Journal of Cardiovascular Electrophysiology

Vol. 25, No. 11, November 2014

Figure 1. Panel A: ECG before ablation with preexcitation. Panel B: Orthodromic AV-reentry tachycardia with a right bundle branch block pattern. Panel C: Successful ablation site with very early local activation time (–35 milliseconds, arrow) before and local endocardial delay after ablation (arrowhead). Panel D: Fluoroscopy demonstrating the long distance between the His catheter and the ablation catheter positioned at the inferior tricuspid annulus. Panel E: Echocardiography demonstrating relevant apical displacement of the septal tricuspid valve leaflet. For a high quality full version of this figure, please see Journal of Cardiovascular Electrophysiology’s website: www.wileyonlinelibrary.com/journal/jce

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Ebstein's anomaly unmasked by accessory pathway ablation.

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