Cardiac arrest in an adolescent with Uhl’s anomaly: Two unusual cardiac arrhythmia mechanisms Jeffrey P. Moak, MD, Gail D. Pearson, MD, ScD, Bradley.

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Cardiac arrest in an adolescent with Uhl’s anomaly: Two unusual cardiac arrhythmia mechanisms Jeffrey P. Moak, MD, Gail D. Pearson, MD, ScD, Bradley Clark, MD, Charles I. Berul, MD, FHRS, Russell R. Cross, MD, Dilip S. Nath, MD HeartRhythm Case Reports Volume 2, Issue 4, Pages 328-333 (July 2016) DOI: 10.1016/j.hrcr.2016.03.004 Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 1 Baseline 12-lead electrocardiogram prior to electrophysiology study. Large P waves were noted in lead V1 and V2. HeartRhythm Case Reports 2016 2, 328-333DOI: (10.1016/j.hrcr.2016.03.004) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 2 Twelve-lead electrocardiograms during the 2 induced arrhythmias. A: Narrow QRS complex tachycardia (cycle length = 240 ms). During this tachycardia 2:1 ventricular-to-atrial (VA) conduction is evident. B: Wide complex tachycardia (left bundle branch block with right axis deviation, cycle length = 235 ms). During this tachycardia, VA dissociation was evident. HeartRhythm Case Reports 2016 2, 328-333DOI: (10.1016/j.hrcr.2016.03.004) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 3 Narrow QRS tachycardia. A: Induction of supraventricular tachycardia (SVT) with 1:2 atrioventricular (AV) response. Following termination of a train of rapid atrial pacing (atrial paced cycle length = 250 ms), a narrow QRS complex tachycardia is induced. The last paced atrial complex results in a double ventricular response, simultaneous AV conduction over the fast and slow AV nodal pathways (arrows). Conduction over the slow AV nodal pathway with a prolonged AH interval facilitates the occurrence of sustained AV nodal reentrant tachycardia (AVNRT) with 2:1 ventricular-to-atrial (VA) conduction. B: SVT with 1:1 VA conduction. Following a different attempt at induction of SVT during rapid atrial pacing, a double antegrade AV conduction response is again observed (1:2 AV conduction) with induction of AVNRT. During this episode of SVT, 1:1 VA conduction is evident. Format of electrocardiographic tracings: 5 surface electrocardiograms, and intracardiac electrograms recorded from the ablation catheter (high right atrium), His bundle catheter, and right ventricular catheter. HeartRhythm Case Reports 2016 2, 328-333DOI: (10.1016/j.hrcr.2016.03.004) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 4 Wide QRS complex tachycardia. A: Induction of wide complex tachycardia (WCT) with rapid atrial pacing. Following the fourth atrial paced event from the left, His-to-ventricular conduction time (HV) block is noted. Gradual HV prolongation is evident on the previous 2 atrial paced beats. The subsequent fifth atrial paced complex is associated with a shortened HV interval and possible pre-excitation. Given the dissociation of atrial and His electrograms from the ventricle on ensuing atrial paced events, it is probable that the tachycardia started on the fourth QRS complex, following the HV block. B: Induction of the WCT during delivery of triple ventricular extrastimuli. The third ventricular paced complex was delivered in the ventricular refractory and does not capture the ventricle. Following the second ventricular extrastimuli 2 beats of atrioventricular node reentry occur, which facilitates the occurrence of the WCT. C: Ventricular pacing at a cycle length = 230 ms in the region of the proximal right bundle branch resulted in concealed entrainment with a post-pacing interval of 15 ms (recovery interval = 264 ms). D: QRS morphology during pacing at the fascicular potential site elicits QRS morphology similar to the WCT (compare with Figure 2B). Format of electrocardiographic tracings is similar to Figure 3. HeartRhythm Case Reports 2016 2, 328-333DOI: (10.1016/j.hrcr.2016.03.004) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 5 Proposed arrhythmia circuit. The proposed arrhythmia circuit suggests antegrade conduction from the right bundle branch (RBB) to the right ventricle (RV) mid septum via a fascicular–ventricular pathway (yellow line). The wide complex tachycardia morphology suggests an origin from the medial right ventricular septum (R wave transition – V4/V5, and right axis deviation). After reaching the moderate band, the wave front reengages the distal RBB and conducts antidromically through ventricular myocardium and back up the proximal right bundle. AVN = atrioventricular node; FV AP = fascicular–ventricular accessory pathway; RA = right atrium. Permission for using the left hand figure granted by @ Kenhub (www.kenhub.com<http://www.kenhub.com>) / Illustration by Y. Koh. HeartRhythm Case Reports 2016 2, 328-333DOI: (10.1016/j.hrcr.2016.03.004) Copyright © 2016 Heart Rhythm Society Terms and Conditions