Lown-Ganong-Levine Syndrome. by Chris Nickson, Last updated January 2, OVERVIEW. bypass close to the AV node connecting the left atrium and the. However, most lack the histopathologic correlation that has been demonstrated for the WPW syndrome. The Lown-Ganong-Levine (LGL). Background: Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia.
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The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular AV node James fiberor between the atria and the His bundle Brechenmacher fiber. Similar features are seen in enhanced atrioventricular nodal conduction EAVNCwith the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria.
A year-old man presented with a history of recurrent narrow-complex and wide-complex ganomg on electrocardiogram ECG. An electrophysiologic study showed an unusually short atrial to His AH conduction interval and a normal His to ventricle HV interval, without a delta wave. Two stable AH intervals coexisted in the same atrial pacing cycle length.
In the recovery curve lwn, this pathway had a flat conduction curve without an AH increase until the last 60 ms, before reaching the effective refractory period. These Gajong changes did not respond to an adenosine challenge. When this pathway became intermittent, there was a paradoxical response to adenosine challenge with conduction via a short AH interval, but without conduction block. Catheter ablation of the AV nodal region resulted in a normalized AH interval, decremental conduction properties, and resulted in a positive response to an adenosine challenge.
In this case of Lown-Ganong-Levine syndrome, electrophysiologic studies supported the role of the accessory pathway of James fibers. Lown-Ganong-Levine syndrome, with the electrocardiographic ECG findings of a short PR interval, a normal QRS complex, and paroxysmal tachycardia, was first described in [ 1 ], and was further characterized by Lown, Ganong, and Levine in [ 2 ].
The proposed pathophysiology of Lown-Ganong-Levine syndome involves accessory pathway connections between the atria and low atrioventricular AV node, as described by James in [ 3 ], or between the atria and the His bundle, as described by Leivne in [ 4 ].
Similar electrophysiologic findings with supraventricular tachycardia SVT and without a delta wave are seen in enhanced atrioventricular nodal synsrome EAVNCwith the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria.
The clinical and electrophysiologic features of a case of recurrent narrow-complex and wide-complex tachycardia on electrocardiogram ECGin a year-old man, are presented. The features of Lown-Ganong-Levine syndrome are compared with those of EAVNC and included analysis of the AV nodal recovery curves obtained before and after adenosine challenge and cryo-ablation.
The findings in this single case, including the short PR and AH interval, may be due to an accessory pathway connection from the atrium to the distal AV node James fiberrather than enhanced atrioventricular nodal conduction EAVNC. A year-old man presented to our institution with a history of recurrent narrow-complex and wide-complex tachycardia. A previous electrophysiologic study at 16 years-of-age demonstrated both antegrade and retrograde dual AV nodal pathways, synddrome pacing-inducible slow and fast AV nodal re-entrant tachycardia.
Extensive radiofrequency ablation of the proximal coronary sinus and the right posteroseptal tricuspid annulus area were unsuccessful at that time. On his recent hospital admission, the electrophysiologic studies showed an extremely short baseline atrial to His AH conduction interval of 22 ms, and a normal His to ventricle HV interval, without a delta wave Figure 2.
At pacing cycle length ms with stable 1: AV nodal Wenckebach block occurred at atrial pacing cycle length of ms. When A1A2 was further decreased, A2H2 gradually increased to ms without a further jump.
The retrograde ventriculoatrial VA conduction had decremental properties.
Ventricular pacing at a pacing cycle length of ms showed a VA conduction of ms with the earliest atrial depolarization at His bundle region. In ventricular extrastimuli, the VA conduction progressively lengthened to ms, then jumped to ms, with the earliest retrograde atrial conduction that shifted to the coronary sinus ostium, which induced a retrograde, slow-antegrade, fast AV nodal re-entry.
With isoproterenol challenge, which is a sympathomimetic for beta-receptors, ventricular pacing induced a sustained fast-slow AV nodal reentrant tachycardia with an AH of 71 ms and HA of ms and the earliest retrograde atrial depolarization was recorded at the area of the ostium of the coronary sinus. Catheter manipulation at the upper mid-septal area incidentally resulted in transient AH prolongation mechanical ablation of the James fiber.
The A1A2-A2H2 plot post-James fiber ablation Figure 3 showed that from A1A2 ms to the A2H2 conduction curve had a decremental property, which was significantly different from that of the pre-ablation curve. From A1A2 to the two recovery curves were superimposable, and this was presumed to be the James fiber effective refractory period.
A repeat adenosine challenge, at the same dose, resulted in transient AV conduction block. However, the James fiber conduction recurred with an intermittent short AH of 33 ms, which was longer than the baseline AH of 22 ms, and a long AH of 50 ms. Adenosine challenge of 0.
Cryoablation pown at the level of lkwn mid-coronary sinus ostium and continued superiorly, finally eliminated the retrograde slow AV nodal pathway at the right lower midseptal area above the coronary sinus ostium. The post-slow pathway ablation recovery curve, with recurrence of the James fiber Figure 3 showed the superimposable recovery curve from A1A2 to ms compared with that of the pre-James fiber ablation curve.
There was no demonstrable retrograde slow AV nodal pathway levie and no inducible AV nodal re-entry with or without isoproterenol challenge. At one-year follow-up, there was no clinical recurrence of tachycardia in this patient. In this syndorme, the clinical and electrophysiologic characteristics were consistent with a diagnosis of Lown-Ganong-Levine syndrome, with a short PR interval, normal QRS complex, without a delta wave, and paroxysmal tachycardia.
This case had the features described by James, as an accessory pathway connection from the atrium to the distal AV node [ 3 syndromme. The findings in this case included an unusually short atrium to His AH conduction interval and a normal His to ventricle HV conduction interval without a delta wave; two different stable AH intervals coexisted at the same atrial pacing cycle length; and in the A1A2-A2H2 recovery curve study, this pathway had a flat conduction curve without AH increase until the lowh 60 ms before reaching its effective refractory period.
When decremental conduction occurred, this pathway showed no response to the adenosine challenge, and when this pathway became intermittent, there was a paradoxical response to adenosine challenge with conduction via a short AH instead of lengthening AH or conduction block. Finally, catheter ablation at the AV nodal region resulted in a normalized AH interval, normal decremental conduction, and resulted ,evine a positive response to adenosine challenge.
In this case, since this pathway had some decremental properties, it is possible that the distal insertion site of this pathway was in the distal AV nodal area James fiber rather than connecting via the atria and the His bundle Brechenmacher fiber [ 34 ]. The James fiber in this patient appeared to be a bystander, which was not a tachycardia substrate. The clinical fast and slow AV nodal re-entrant tachycardia utilized an antegrade normal AV nodal pathway and a retrograde slow AV nodal pathway.
Therefore, after the James fiber recurrence, further ablation was ganon pursued. Instead, the retrograde slow AV nodal pathway was eventually ablated successfully at the right lower mid septal area above the coronary sinus ostium.
When the James fiber became refractory at A1A2 of mssynxrome conduction continued through the AV nodal pathway with similar pre- and post-ablation curves, that were almost superimposable. Comparison of the post-James fiber ablation recovery curve with that of the post-slow pathway ablation curve with James fiber recurrence, it was noted that when the A1A2 was less ganogn ms, the post-slow pathway ablation curve fast AV nodal conduction curve deviated downward from that of the pre-James fiber ablation curve slow pathway conduction curve.
Therefore, the pre-James fiber ablation curve was a hybrid of a James fiber and a slow AV nodal pathway conduction curve; the post-James fiber ablation curve was a hybrid of fast and slow AV nodal conduction curve, and the post-slow pathway ablation curve was a hybrid of the James fiber and fast AV nodal conduction levind.
The diagnosis of EAVNC implies that the underlying pathology is tanong to a distal atrial insertion to the AV node or the fast pathway input to the AV node interposed by less AV nodal tissue than normal, before entering the His bundle. If this were the case, when this EAVNC was injured during catheter manipulation and by catheter ablation with intermittent AH prolongationadenosine should have resulted in a further lengthening of the AH or caused AV block. Instead, the normal AV nodal pathway was blocked, resulting in the paradoxical response of constant short AH intervals.
The differential diagnosis between the small AV node showing properties of EAVNC and the presence of the anatomically independent James fiber can be quite difficult.
If the James fiber had retrograde AV conduction, it would have been possible to also evaluate the anatomical difference in the atrial insertion site between the James fiber and the fast AV nodal pathway.
Unfortunately, this case showed no evidence of retrograde AV conduction through the James fiber. Tachycardia is one of the features of Lown-Ganong-Levine syndrome.
Similar to Wolff-Parkinson-White syndrome, Lown-Ganong-Levine syndrome can result in serious cardiac arrhythmias, atrial fibrillation, several syncope episodes, and even sudden death [ 2 ]. Ina clinical study of children and young adults included cases of sudden death, in which out of ten cases of ventricular pre-excitation 3. In the two cases of Lown-Ganong-Levine syndrome, one case had a hypoplastic atrioventricular node, likely to have been caused by EAVNC, and the other had Brechenmacher fibers ganobg tracts ; of the cases of Wolff-Parkinson-White syndrome, sudden cardiac death was related to a very short ante-grade effective refractory period of the accessory pathway [ 8 ].
Since the tachycardia in this case was caused by fast and slow AV nodal re-entrant tachycardia and the antegrade effective refractory period of the James fiber was relatively long msin retrospect, there may have been no need to gsnong the James fiber in this case. However, attempted cryoablation ofthe James fiber proved its presence. Since the James fiber is close to the normal AV nodal tissue, cryoablation, with its reversibility in case of an adverse event of AV block, should be used if necessary.
This single case report is of Lown-Ganong-Levine syndrome associated with accessory pathway James fiber conduction, but this single case does not attempt to apply this finding to the cause in all cases of this syndrome. With the increasing use of the cardiac electrophysiologic studies and catheter ablation in the evaluation of patients with cardiac pre-excitation syndromes, it is likely that more cases of Lown-Ganong-Levine syndrome will be studied.
An awareness of the clinical and electrophysiologic features of Lown-Ganong-Levine syndrome will assist the cardiologist and electrophysiologist in making the correct diagnosis and may add further insight into the pathophysiology of this syndrome. National Center for Biotechnology InformationLpwn. Am J Case Rep. Published online Mar Find articles by Juanita Hunter. Find articles by Emmanouil Tsounias.
Find sydrome by John Cogan. Find articles by Ming-Lon Young. Author information Article notes Copyright and License information Disclaimer. Received Aug 22; Accepted Dec Male, 17 Final Diagnosis: Background Lown-Ganong-Levine syndrome, with the electrocardiographic ECG findings of a short PR interval, a normal QRS complex, and paroxysmal tachycardia, was first described in [ 1 ], and was further characterized by Lown, Ganong, and Levine in [ 2 ].
Case Report A year-old man presented to our institution with a history of recurrent narrow-complex and wide-complex tachycardia. Open in a separate window.
Discussion In lveine case, the clinical and electrophysiologic characteristics were consistent with a diagnosis of Lown-Ganong-Levine syndrome, with a short PR interval, normal QRS complex, without a delta wave, and paroxysmal tachycardia. Conclusions This single case report is of Lown-Ganong-Levine syndrome associated with accessory pathway James fiber conduction, but this single case does not attempt to apply this finding to the cause in all cases of this syndrome.
Without deformation of the ventricular complex Arch Mal Coeur. Morphology of the human atrioventricular node, with remarks pertinent to its electrophysiology. Analysis of anterograde and retrograde fast pathway properties in patients with dual atrioventricular nodal pathways. Observations regarding the pathophysiology of the Lown-Ganong-Levine syndrome. Dougherty A, Naccarelli G. Characteristics of ventriculoatrial conduction in patients with enhanced ldvine nodal conduction.
Characteristics of atrioventricular conduction and the spectrum of arrhythmias in Lown-Ganong-Levine syndrome. Ventricular pre-excitation in children and young adults. Atrial myocarditis as a possible trigger syhdrome sudden death. Support Center Support Center.