Circulation: Arrhythmia and Electrophysiology CHALLENGE OF THE WEEK

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A 14-year-old girl with Wolff-Parkinson-White syndrome and recurrent paroxysmal palpitations due to atrioventricular reentry tachycardia had undergone two prior failed left lateral accessory pathway ablations at another institution and is now referred to you. Her electrocardiogram is shown in Figure 1. She is brought to the electrophysiology laboratory and orthodromic atrioventricular reentry tachycardia is inducible. Figure 2 shows the fluoroscopic catheter locations and correlating intracardiac electrograms. Ventriculoatrial conduction time is constant with ventricular pacing at different cycle lengths. Figure 3 shows a parahisian pacing maneuver. What can you say about the accessory pathway? Figure 1. Twelve lead electrocardiogram. Noheria A. Challenge of the Week: May 7 th Question

Circulation: Arrhythmia and Electrophysiology CHALLENGE OF THE WEEK RAO LAO Figure 2A. Fluroscopy. Right anterior oblique and left anterior oblique fluoroscopic views to show catheter positions. th Question {Author Challenge the Week: Question. Circ Arrhythm Electrophysiol. 2018. http://xxxxxx Noheria Name}, A. Challenge of theofweek: May 7{Date}

Figure 2B. Intracardiac electrograms. Correlating intracardiac electrograms. P1 ART is the arterial blood pressure tracing; I, II, avf and V1 represent surface electrocardiogram leads; RVa, right ventricular apex; HRA, high right atrium; HIS 4 to HIS 1, His bundle electrograms proximal to distal; ABL p, ablation catheter proximal; ABL d, ablation catheter distal; CS 19,20 to CS 1,2, coronary sinus catheter proximal to distal. Noheria A. Challenge of the Week: May 7 th Question

Figure 3. ParaHisian pacing maneuver. Noheria A. Challenge of the Week: May 7 th Question

Answer Options: A. There is evidence for more than one accessory pathway. B. There is evidence for accessory pathway slant from atrial insertion at CS 7,8 and ventricular insertion at CS 1,2. C. The ventriculoatrial conduction is via the atrioventricular node. D. Ablation should be performed at the location of the ablation catheter (ABL d) in Figure 2. E. Left atrial appendage should be mapped. {Author Noheria Name}, A. Challenge Challenge of the of Week: the Week: May 7{Date} th Question Question. Circ Arrhythm Electrophysiol. 2018. http://xxxxxx

ANSWER TO May 7 th QUESTION E. Left atrial appendage should be mapped Explanation Preexcitation on electrocardiogram: The 12 lead electrocardiogram in May 7th Question shows preexcitation localizing the accessory pathway to the lateral/superolateral mitral annular region. 1 Antegrade atrioventricular conduction: The electrograms in sinus rhythm show evidence of preexcitation with earliest ventricular electrograms seen as similarly early farfield signals on coronary sinus (CS) 5 6, 7 8, 9 10 and ablation distal (ABLd) (Figure 1). We do not see a clear near field accessory pathway potential or a singularly early near field ventricular electrogram to be considered the ventricular insertion site for ablation (Option D is wrong). Retrograde ventriculoatrial conduction: Retrograde atrial activation sequence The parahisian pacing maneuver (Figure 2)shows a constant retrograde atrial activation sequence. The absence of more than one activation sequence suggests retrograde activation through the tracing occurring using the same connection. Activation occurring over a discrete accessory pathway across the annulus to the atrial myocardium would be expected to have a radial spread from an early site, with near field atrial electrograms on the CS catheter arranged in a chevron pattern. The earliest atrial electrograms seen in Figure 2 are similarly early far field potentials on CS 1 2, 3 4, 5 6 and 7 8; closely followed by near field activation on the same electrodes. Multiple electrodes activated at about the same time with far field low frequency component preceding the near field suggests an activation wavefront propagating from a distant site to all the early recording sites. Focal early activation locally at any of the sites would have been distinctly earlier than the neighboring sites. 2 Absence of ventriculoatrial fused electrograms The local ventricular and atrial electrograms are spaced out without a fused ventriculoatrial signal usually seen with accessory pathway conduction. The separation of the local ventricular and atrial signals (double potential) has the following differential diagnosis: Slow conduction If this accessory pathway has a long conduction time, this will separate out the local ventricular and the local atrial signal by the time is takes to conduct across the accessory pathway. This is similar to the atrio His (AH) interval seen with slow conduction across the atrioventricular node. Accessory pathway slant If the accessory pathway is slanted going across the atrioventricular annulus e.g. with ventricular insertion close to CS 7 8 and an atrial insertion close to CS 3 4, the ventriculoatrial conduction wavefront from will have to take a zig zag

route (Figure 3). This introduces a longer conduction path A and B capture the left bundle, while loss of direct leftbundle capture in complex C delays conduction time to the length and physiological slowing of conduction velocity at sites of curvature. 3,4 Option B is incorrect because the atrium. This suggests ventriculoatrial conduction ventricular insertion of the accessory pathway at CS1 2 is dependent on the conduction system, in this case incongruous with the earliest antegrade ventricular dependent on the left bundle branch to get quickly to the electrograms seen on CS 5 6 to CS 9 10; and similarly atrial left ventricular free wall where the accessory pathway insertion at CS 7 8 does not explain the similarly early connects to the atrium.6 The atrial activation sequence is retrograde electrograms on CS 1 2 to CS 5 6. eccentric (septal activation seen on HIS4 occurs after CS 1 Absence of conduction (conduction block) In this case 2, 3 4, 5 6 and 7 8 excluding conduction through there is no direct conduction from the local ventricular atrioventricular node (option C is incorrect). Absence of electrogram to the local atrial electrogram across an direct capture of the local ventricular myocardium in accessory pathway, but conduction from the ventricle addition to the left conduction system at the parahis to the atrium occurs at some remote site e.g. the aortic pacing site in complex A as compared to complex B does sinuses of Valsalva not being mapped by the available not result in any change in stimulus to atrial conduction intracardiac electrodes. The local ventricular and local time and activation sequence, demonstrating the activation atrial activation is then just bystander activation does not use a septal accessory pathway. 6 (pseudointerval). 5 Putting it all together: In light of the above points this case ParaHisian pacing maneuver: The pace stimuluses A, B and can be explained without having to invoke more than one C in Figure 2 respectively generate QRS complexes accessory pathway (option A is incorrect). There is a suggesting selective left bundle branch capture (stimulusto QRS delay and typical right bundle branch morphology), pathway in the left superolateral mitral annular region. bidirectionally conducting atrioventricular accessory non selective left bundle/his bundle capture along with However, the temporal separation between the ventricular local myocardial capture (narrower QRS), and local and atrial electrograms and the absence of the CS myocardial capture alone (wider QRS). The atrial activation electrograms arranged in a chevron pattern, but with sequence is unchanged excluding participation of two multiple electrodes activated about simultaneously would separate ventriculoatrial conduction pathways. Complexes suggest the atrioventricular connection is not in proximity

route (Figure 3). This introduces a longer conduction path A and B capture the left bundle, while loss of direct leftbundle capture in complex C delays conduction time to the length and physiological slowing of conduction velocity at sites of curvature. 3,4 Option B is incorrect because the atrium. This suggests ventriculoatrial conduction ventricular insertion of the accessory pathway at CS1 2 is dependent on the conduction system, in this case incongruous with the earliest antegrade ventricular dependent on the left bundle branch to get quickly to the electrograms seen on CS 5 6 to CS 9 10; and similarly atrial left ventricular free wall where the accessory pathway insertion at CS 7 8 does not explain the similarly early connects to the atrium.6 The atrial activation sequence is retrograde electrograms on CS 1 2 to CS 5 6. eccentric (septal activation seen on HIS4 occurs after CS 1 Absence of conduction (conduction block) In this case 2, 3 4, 5 6 and 7 8 excluding conduction through there is no direct conduction from the local ventricular atrioventricular node (option C is incorrect). Absence of electrogram to the local atrial electrogram across an direct capture of the local ventricular myocardium in accessory pathway, but conduction from the ventricle addition to the left conduction system at the parahis to the atrium occurs at some remote site e.g. the aortic pacing site in complex A as compared to complex B does sinuses of Valsalva not being mapped by the available not result in any change in stimulus to atrial conduction intracardiac electrodes. The local ventricular and local time and activation sequence, demonstrating the activation atrial activation is then just bystander activation does not use a septal accessory pathway. 6 (pseudointerval). 5 Putting it all together: In light of the above points this case ParaHisian pacing maneuver: The pace stimuluses A, B and can be explained without having to invoke more than one C in Figure 2 respectively generate QRS complexes accessory pathway (option A is incorrect). There is a suggesting selective left bundle branch capture (stimulusto QRS delay and typical right bundle branch morphology), pathway in the left superolateral mitral annular region. bidirectionally conducting atrioventricular accessory non selective left bundle/his bundle capture along with However, the temporal separation between the ventricular local myocardial capture (narrower QRS), and local and atrial electrograms and the absence of the CS myocardial capture alone (wider QRS). The atrial activation electrograms arranged in a chevron pattern, but with sequence is unchanged excluding participation of two multiple electrodes activated about simultaneously would separate ventriculoatrial conduction pathways. Complexes suggest the atrioventricular connection is not in proximity

to the multielectrode CS catheter circling around the left ventricular outflow and the left ventricular inflow atrioventricular annulus. The atrioventricular annulus is the respectively. The appendages and the ventricles however predominant site of atrioventricular connections, but the develop from embryologically distinct and separate tissues left atrial appendage overhangs the basal left ventricle and and their respective myocardium is separately covered by is another potential site for connection between the atrial the visceral pericardium precluding to and fro conduction. any ventricular myocardium, and should be mapped However, acquired accessory pathways have been (Option E is correct). This would also explain the failure of described from either of the appendages to the adjacent prior two ablation procedures. Intracardiac ventricular myocardium. This may be hypothesized to occur echocardiography guided mapping within the left atrial with healing after a localized myopericarditis and appendage showed retrograde electrograms earlier than sometimes is acquired in deposition disorders like Fabry s the CS catheter and ablation within the appendage cardiomyopathy. Right atrial appendage to right ventricular eliminated accessory pathway conduction. outflow tract. connections can develop in patients with the Non annular atrioventricular connections: The atrial and classic right atrial appendage right ventricular outflow tract ventricular myocardium are contiguous during embryonic Fontan. Similarly, the venous myocardium from the CS development but are separated except at the extending into a CS diverticulum or the coronary veins can atrioventricular node with development of the fibrous develop an epicardial connection to the adjacent annular tissue. Failure of this process is thought to be ventricular myocardium. Other unusual sites for responsible for most accessory pathways located on the atrioventricular connection could be through the annuli. Other than the atrioventricular annuli, the right and aortomitral continuity and conceivably from the noncoronary cusp to the adjacent atrial left atrial appendages are in contact with the right muscle. References: 1. Arruda MS, McClelland JH, Wang X, Beckman KJ, Widman LE, Gonzalez MD, Nakagawa H, Lazzara R, Jackman WM. Development and validation of an ECG algorithm for identifying accessory pathway ablation site in Wolff Parkinson White syndrome. J Cardiovasc Electrophysiol. 1998;9:2 12. 2. Noheria A. April 16th Question. Circ Arrhythm Electrophysiol. 2018;11:e006443. 3. Otomo K, Gonzalez MD, Beckman KJ, Nakagawa H, Becker AE, Shah N, Matsudaira K, Wang Z, Lazzara R, Jackman WM. Reversing the direction of paced ventricular and atrial wavefronts reveals an oblique course in accessory AV pathways and improves localization for catheter ablation. Circulation. 2001;104:550 556. 4. Cabo C, Pertsov AM, Baxter WT, Davidenko JM, Gray RA, Jalife J. Wave front curvature as a cause of slow conduction and block in isolated cardiac muscle. Circ Res. 1994;75:1014 1028. 5. Asirvatham SJ. Diagnostic Maneuvers Commonly Used in the Electrophysiology Laboratory: Understanding the Rationale, Techniques, and Interpretation. In: Mayo Clinic Electrophysiology Manual. Oxford, UK: Oxford University Press; 2013. p. 85 128. 6. Sheldon SH, Li H K, Asirvatham SJ, McLeod CJ. Parahisian pacing: technique, utility, and pitfalls. J Interv Card Electrophysiol. 2014;40:105 116.

Figure 1. Intracardiac electrograms during sinus rhythm. Similarly early far field ventricular activation is seen on electrograms at CS 5 6, 7 8, 9 10 and ABLd. There is no definite earliest ventricular electrogram or accessory pathway potential as the target of ablation on the ABLd electrogram (arrow). P1 ART is the arterial blood pressure tracing; I, II, avf and V1 represent surface electrocardiogram leads; RVa, right ventricular apex; HRA, high right atrium; HIS 4 to HIS 1, His bundle electrograms proximal to distal; ABL p, ablation catheter proximal; ABL d, ablation catheter distal; CS 19,20 to CS 1,2, coronary sinus catheter proximal to distal.

Figure 2. ParaHisian pacing maneuver. Complexes A, B and C respectively show selective His/left bundle branch (LBB) capture, His/LBB + ventricular capture and pure ventricular capture. The double headed arrows show the fixed ventriculoatrial conduction time for complexes A, B and C. A, atrial; AEGM, atrial electrograms.

Figure 3. Putative oblique accessory pathway to explain separation between ventricular and atrial electrograms during retrograde conduction in Figure 2. Overlaid on the left anterior oblique (LAO) fluoroscopic projection, the location of the early antegrade ventricular electrograms (V EGMs) is marked with purple and the location of the retrograde early atrial electrograms (A EGMs) is marked in green. The zig zag course (dashed arrow) taken by the wavefront to go from ventricle to the atrium introduces temporal delay due to the longer course and the slowing of conduction on account of wavefront curvature. CS, coronary sinus.