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Arrhythmia Rounds
Section Editor: George J. Klein, M.D.

Paroxysmal High-Grade Atrioventricular Block and Syncope in a Previously Healthy Child: What is the Mechanism?
RONALD J. KANTER, M.D.,∗ SALIM IDRISS, M.D., Ph.D.,∗ ´ and JORGE GONZALEZ ZUELGARAY, M.D.†
From the ∗ Duke University School of Medicine, Durham, North Carolina, USA; and †Instituto Argentino de Diagn´ ostico y Tratamiento; Buenos Aires, Argentina

Case Presentation This 11-year-old previously healthy female began having syncopal episodes at rest and during exertion. Baseline ECG (PR interval = 155, QRS duration = 85, and QTc = 389 ms) and echocardiogram were normal. During 24-hour ambulatory heart rhythm monitoring, there were several episodes of paroxysmal high grade AV block not associated with symptoms (Fig. 1A). During block, the maximum RR interval was 2.8 seconds, when the P wave rate was stable at 105 bpm. A diagnostic electrophysiologic study was performed under propofol anesthesia. Standard quadripolar electrode catheters having 2-5-2 interelectrode spacing were placed in the high right atrium, right ventricular apex, and His bundle regions. At baseline, a “split His bundle” electrogram (denoted as H-H’) was always present during sinus rhythm and atrial pacing, with a constant H’V interval of 35–38 ms. During atrial extrastimulus testing and incremental atrial pacing, the AH and AH’ intervals increased similarly (Fig. 1B) with a nearly constant H-H’ and without classic criteria for dual AV nodal physiology. What do you think is the mechanism of block? Discussion Adenosine (200 mcg/kg) resulted in AH prolongation, followed by H-H’ block, followed, in turn, by A-H block (Fig. 2A). During sustained reflex vagotonia from phenylephrine infusion 0.5 mcg/kg/min, a sequence of A-H block and A-H’-V was demonstrated (Fig. 2B), but high-grade AV block was not observed. At no time was there H’-V prolongation, H’-V block, a change in QRS axis, or occurrence of bundle branch block. Two His bundle-like electrograms were present during ventricular pacing, as well. Because their specific correspondence to H and H’ is not proved, and to avoid confusion, they are designated as Ha and Hb. During ventricular extrastimulus testing, a V-Ha-A-Hb sequence was always present.
J Cardiovasc Electrophysiol, Vol. 21, pp. 585-587, May 2010. No disclosures. Address for correspondence: Ronald J. Kanter, M.D., Duke University Medical Center; Box 3090, Durham, NC 27710. Fax: 919-668-6122; E-mail: kante001@mc.duke.edu doi: 10.1111/j.1540-8167.2009.01676.x

Decremental retrograde conduction was mostly at the Ha-A level. Adenosine 200 mcg/kg increased VHa-to-A conduction time, followed by A-to-Hb block for 1 beat, followed, in turn, by V-Ha-to-A block (Fig. 3A). No reentrant tachycardias or even “echo beats” could be induced at baseline or during isoproterenol infusion at 0.05 mcg/kg/min. A single chamber ventricular pacemaker was placed, and the patient has remained asymptomatic for 9 years. Commentary High-grade AV block is defined as the abrupt onset of ≥ 2 consecutive nonconducted P waves, without ventricular escape, in the context of a normally-conducting, nontachycardic atrial rhythm. It is an uncommon rhythm and has

Figure 1. (A) Rhythm strip from ambulatory monitor showing paroxysmal high-grade AV block. (B) Intracardiac electrograms during incremental atrial pacing, recorded at 200 mm/s. The numbers represent the AH/HH’/H’V intervals, in ms. Surface leads III, V1, and V6; and intracardiac channels from the high right atrium (HRA), proximal His bundle region (His), and right ventricular apex (RVA) are illustrated. PCL = pacing cycle length (in ms.).

12-23 lengthening of HV interval at critical H1H2 coupling intervals. more proximal) conduction into the His electrogram designated as H and slower (or.5 mcg/kg/min. V6. always follows an atrial electrogram. The curved. dotted arrow represents reentry within AV nodal pathways. The observation of separate A-H and A-H’ block but never A-H-V conduction supports the notion that H’ does not conduct distally. retrograde conduction exists only through a single His bundle (Ha) and a slowly conducting AV nodal output (assuming absence of any accessory atrioventricular pathway). Hb. Although we still consider as possible a more conventional schema of longitudinal dissociation of the entire AV axis in our patient (illustrated in Fig. The curved. May 2010 Figure 2. Surface lead V6. always in association with other conduction findings: change in QRS axis and/or morphology (if not complete bundle branch block). this phenomenon has been demonstrated by multiple authors. 21. Because the second His bundle. H’. in ms. Similarly. See text for explanation. and ladder diagram during ventricular pacing at cycle length 500 ms. 5. Bl = conduction block. Based upon Figures 1B and 2. with the His bundle identified as H’ having completely normal conduction properties. in which longitudinal dissociation of His bundle conduction is based upon anatomic duplication of this structure. following administration of adenosine 200 mcg/min. and never showed a change in QRS morphology. and ladder diagram during atrial pacing at cycle length 420 ms. Bl = conduction block. 3C). the possible conduction pathways in this patient suggested by electrophysiological testing.26 Our patient showed a persistent H-H’ complex (except during AV nodal blocking maneuvers). it must be anterogradedly activated.1-10 In our patient. . intracardiac testing suggested longitudinal dissociation of at least a portion of the specialized AV conduction system to account for her symptoms.24 splitting of the His bundle electrogram only at specific H1-H2 intervals. always had a normal H’V interval. (B) In the presence of longitudinal dissociation of both the AV node and His bundle. (B) Intracardiac electrograms recorded at 200 mm/s. The numbers represent the A-H/H-H’/H’-V intervals. No.25 and/or infraHisian block. perhaps. proximal His bundle region (His). dotted arrow represents reentry within AV nodal pathways.22. Sano first explored the concept of longitudinal dissociation of the His bundle in 1974.17. and right ventricular apex (RVA) are illustrated. (A) Intracardiac electrograms recorded at 200 mm/s and ladder diagram during atrial pacing at cycle length 340 ms. never had H’-V block. perhaps.11 Since then. The numbers represent HaA/A-Hb intervals. See the text for further discussion. following administration of adenosine 200 mcg/kg. See text for explanation. the above reasons suggest the presence of a unique conduction arrangement (Fig. (A) Intracardiac electrograms recorded at 200 mm/s. The alternate possibility—that Figure 3. See text for explanation. 0. (C) Alternate explanation for possible conduction pathways in this patient. and intracardiac channels from the high right atrium (HRA).586 Journal of Cardiovascular Electrophysiology Vol.19. 3B).15.22. more distal) conduction into the His bundle. under the influence of phenylephrine infusion. in which longitudinal dissociation of the His bundle is based upon separate His bundle-like structures. Surface lead V1. and intracardiac channels from the high right atrium (HRA). proximal His bundle region (His). we speculate that longitudinal dissociation of the AV node manifests as faster (or. and right ventricular apex (RVA) are illustrated. been reported in association with a variety of cardiac and noncardiac conditions.

Rosen K. Delise P. 2. refractory by the mechanism of repetitive concealed electrotonic conduction. Cor Vasa 1989. when there is preferential conduction through the faster AV nodal input and subsequently to the His bundle. 17. Tamura M. Stein KM. preventing anterograde conduction into the His bundle.45:16401644. Allibardi P. Iesaka Y: Longitudinal dissociation in the his bundle: A possible mechanism of two distinct cycle lengths in atrioventricular reentrant tachycardia. only “vagally” related to the heart. 20. Akiyama J. could also cause concealed conduction into the distal portion of the slower AV nodal input. Pacing Clin Electrophysiol 1982. Lerman BB: AV nodal-HisPurkinje reentry: A novel form of tachycardia.52:483-486. Tsujimura Y. Pacing Clin Electrophysiol 1997. which conducts to a slowly conducting AV nodal output in Figure 3A is the same His bundle (H’) that conducts anterogradedly from the AV node to the ventricle in Figure 2.117:1801-1803.6:400-409. Morena H. Clinical and experimental evidence of longitudinal dissociation in the pathologic his bundle. J Electrocardiol 2002. Kawamura Y.19:325330.73:536-537. Markowitz SM.56:996-1006. due to alternating Wenckebach periodicity at more than 1 anatomic level. Epilepsia 2004. Aust N Z J Med 1985. 2B). in the realm of speculation. Ito T.Kanter et al. Arch Mal Coeur Vaiss 1981. H’. sudden and isolated AV nodal block due to enhanced parasympathetic tone is the simplest explanation. A new mechanism for intermittent intraventricular blocks? G Ital Cardiol 1984. Rakovec P. Ha. Tsuji Y. This mechanism would be facilitated by longitudinal dissociation of the AV node. Di Pede F. Okamoto Y. Wirth R. 7. Katoh T. (1) If the scheme in Figure 3B is operative. Brezinski D. Olsen S. but blocks in the slower AV nodal input that ordinarily conducts to the His bundle. Smith TW. Takada G: Abrupt aggravation of atrioventricular block and syncope in hypertrophic cardiomyopathy. Arthritis Rheum 1989. Piccolo E: Mid-diastolic intraventricular functional block. H. Bonso A. Katoh T. Kinoshita S. 19. Tanabe Y. Int Heart J 2005. Prognostic significance of precordial ST segment depression. Rubio Sanz R. a case report. high-grade AV block would result.20:2016-2018. Ziegelstein RC: A patient with syncope. H’. Marumo F. . Zehender M. Circulation 1977. Gierke LW.36:81-84.14:1-11. Sasaki Y: Double ventricular response caused by longitudinal dissociation in the his bundle.15:681-684. Kinoshita S. Iesaka Y. Fenster PE. Raviele A. Arch Dis Child 1995. Walsh RL. but is not supported by this patient’s rhythm strips: 4 consecutive nonconducted P waves bracketed by beats having normal PR intervals and no periodicity to the P-QRS relationships. This mechanism would not require repetitive concealment between the 2 AV nodal inputs. Katoh T. Marcus FI: Transient heart block associated with head trauma. 14. 13.15:248261. Isomoto S. one could propose the following: depending upon the potential for interactions between the distal AV nodal components that conduct to the separate His bundles. Circulation 2001. Ogawa Y. 15. Guarnieri T. Gosselin AJ. Kamiyama A: Evaluation of concept of longitudinal dissociation of His bundle. 5. 12. H. 8. Ahmad M. J Cardiovasc Electrophysiol 1995. El-Sherif N. Hochberg MC. Chest 1985. Altenmuller DM. Electrophysiologic and histopathologic study. Then. 16. Multiblock. Ogahara S. Sebag C. Mooto G. Chandler FW: Lyme carditis. Pacing Clin Electrophysiol 1996. H. Katoh T. Gorelkin L. J Electrocardiol 2003. de Boisgelin X: Improvement and normalisation of the QRS complex by stimulation of the bundle of His in complete left branch block. The mechanism of high-grade AV block observed in our patient is unexplained. Sawanobori T. Engelstein ED. Arch Inst Cardiol Mex 1982. Tsujimura Y. Since there is permanent HV block through the His bundle. Sano T. 22. Tsujimura Y.74:705717.32:1170-1174. 25. Sasaki Y: Atypical atrioventricular Wenckebach periodicity caused by conduction through triple atrioventricular junctional pathway as a probable mechanism. Watanabe Y: Double His deflections caused by intra-Hisian conduction disturbance with longitudinal dissociation. Amay-Y-Leon F. Sato N. Am J Cardiol 1984. Scherlag BJ. Almazan Ceballos A. Lister JW: Intrahisian functional bundle branch block. Marine JE. Reznick JW. Sasaki Y: Alternating reversed wenckebach periodicity: Concealed electrotonic conduction as a possible mechanism. Puech P. J Electrocardiol 2003. Davy JM: [Fragmenting of the His potential after atrial stimulation]. Arch Mal Coeur Vaiss 1979. Nishimura M. Lajovic J. Harada K. properly timed anterograde conduction into the blind-ending His bundle. 6. Taylor AJ. H. Longitudinal dissociation and concealed electrotonic conduction involving fast and slow inputs to the AV node has been previously proposed as a mechanism of AV block. Centurion OA. Hayano M. Kitaoka T. Hasebe N. Grolleau R. Ockner SA: Unusual cardiac complications of Wegener’s granulomatosis.88:745-748. Lazzara R. Bundle branch block due to asynchronous conduction within the His bundle in man. H’ (similar to the 4th beat from Fig.46:737-744. Najjar SS.53:368-370. Aonuma K. Two other mechanisms should be considered. Kinoshita S. and review of the literature.35:69-74.5:667-674. Cataldo R. Arrhythmia Rounds 587 there was retrograde V-to-Hb conduction (with extremely long conduction time)—was refuted by V-to-Hb block by adenosine. D’Este D. Pariente P. Braunstein DB. Kikuchi K: A case of alternating bundle branch block in combination with intra-Hisian block. Enoki M. 23. if the scheme put forth in Figure 3C is accurate. 26. Desroches W: [Longitudinal dissociation of the bundle of His and infra-His block]. Kato T. References 1.27 (2) Again. Rozanski JJ. Though uncommon. Nogami A. Umezawa S. 9. Jagger JD: Long term follow-up of inferior myocardial infarction.28:262-264. Eldadah ZA. subsequent atrial beats could repetitively penetrate into the slower AV nodal input.31:145-148. Int J Cardiol 1994. Shiokoshi T. Korenaga M. a fortuitously timed atrial impulse that conducts through the faster AV nodal input and into the His bundle. is a known cause of high-grade AV block. Jpn Heart J 1974. Konoe A. Hiroe M. Pascotto P. Circulation 1978. Bilazarian SD. Smith CR. Monahan KM: High-grade atrioventricular block caused by his-purkinje injury during contrast left ventriculography. Wolfson PM.72:815-824. Dolenc M: Longitudinal dissociation in the His bundle. Am J Med 1986. Wyndham C: Normalization of bundle branch block patterns by distal His bundle pacing. Molinero Somolinos F. Schonfield C. Provost TT: High-grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies. 18. It is logical that the sole retrogradely conducting His bundle electrogram. 4. 11. 21. 3.104:E77-E78. 10.57:473-483. Inada M. Freedman RA: Atrioventricular block occurring late after heart transplantation: Presentation of three cases and literature review. Yano K: Electrophysiologic demonstration of anterograde fast and slow pathways within the His bundle in patients with normal intraventricular conduction. could render the functional His bundle. Chest 2000.81:923-927. Narula OS: Longitudinal dissociation in the His bundle.44:251-260. 27.36:73-79. Suzuki T. Shimizu A. J Trauma 1988. Schulze-Bonhage A: High-grade atrioventricular block triggered by spontaneous and stimulation-induced epileptic activity in the left temporal lobe. 24. Ogura Y. Schiavone WA. Pinakatt T. Laurent M.

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