Lown-Ganong-Levine syndrome

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Lown-Ganong-Levine syndrome Microchapters

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Lown-Ganong-Levine Syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Treatment

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Usman Ali Akbar, M.B.B.S.[3]

Synonyms and Keywords: Lown-Ganong-Levine Syndrome, LGL syndrome, Pre-excitation syndromes, Short PR Normal QRS Complex Syndrome, Clerc-Lévy-Cristesco syndrome, Coronary nodal rhythm syndrome, Short PQ interval syndrome, Short P-R syndrome.

Overview

Lown-Ganong-Levine syndrome (LGL) is actually a pre-excitation syndrome with EKG findings including short PR interval, narrow or normal QRS complex, and a normal P wave. It is caused by the presence of accessory bundles of fibers known as James fibers which lead to the development of abnormal conduction pathways. The LGL syndrome was named after Bernard Lown, William Francis Ganong, and Samual Levine who described it in 1952. Patients of LGL usually present with a history of palpitations, lightheadedness, shortness of breath, and sometimes chest pain. There is an increased risk of tachyarrhythmias and syncope. EKG is the principal modality of investigation for establishing a diagnosis. Usually, antiarrhythmics are given to prevent the development of tachyarrhythmias but recently radiofrequency ablation of the accessory pathways has been the mainstay of treatment with a good prognosis.

Historical Perspective

Historical timeline of LGL Syndrome
Year Description
1938
1946
1952
  • The Lown-Ganong-Levine (LGL) pattern was described in 1952 by Bernard Lown, William Francis Ganong, and Samual Levine.
1961-1974
  • In 1961 and subsequently, in 1974, an anatomic pathway was identified and reported by James and Brechemacher respectively.
Historic Timeline of LGL Syndrome

Classification

Accessory Pathway Description
James fibers
Brechenmacher fibers
Intra-nodal bypass tracts

Pathophysiology

LGL Syndrome associated bypass tracts

Causes

Differentiating Lown-Ganong-Levine Syndrome from other Diseases


Arrhythmia Rhythm Rate P wave PR Interval QRS Complex Response to Maneuvers Epidemiology Co-existing Conditions
LGL Syndrome
  • Present
Atrial fibrillation (AFib)[6][7]
  • Absent
Atrial flutter[8]
Atrioventricular nodal reentry tachycardia (AVNRT)[9][10][11][12]
  • Regular
Multifocal atrial tachycardia[13][14]
Paroxysmal supraventricular tachycardia
  • Regular
  • 150 and 240 bpm
  • Absent
  • Hidden in QRS
  • Absent
Premature atrial contractrions (PAC)[15][16]
  • Upright
  • Usually narrow (< 0.12 s)
_
Wolff-Parkinson-White Syndrome[17][18]
  • Regular
Ventricular fibrillation (VF)[19][20][21]
  • Absent
  • Absent
Ventricular tachycardia[22][23]
  • Regular
  • > 100 bpm (150-200 bpm common)
  • Absent

Epidemiology and Demographics

Age

  • There is currently insufficient data regarding age predilection of LGL syndrome.

Gender

  • There is currently insufficient data regarding gender predilection of LGL syndrome as the LGL pattern is not associated with an increased incidence in one particular sex.
  • However, Lown in 1952 reported 70.9% of the 34 cases in women.[1]

Race

Risk Factors

Natural History, Complications and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

Lown-Ganong-Levine syndrome ECG features. [1]

History and Symptoms

Physical Examination

Imaging Findings

ECG

ECG showing LGL syndrome with short PR interval, narrow QRS complex, and normal P waves. Source: LITFL

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 LOWN, BERNARD; GANONG, WILLIAM F.; LEVINE, SAMUEL A. (1952). "The Syndrome of Short P-R Interval Normal QRS Complex and Paroxysmal Rapid Heart Action". Circulation. Ovid Technologies (Wolters Kluwer Health). 5 (5): 693–706. doi:10.1161/01.cir.5.5.693. ISSN 0009-7322. PMID 14926053.
  2. 2.0 2.1 Manning, G W (1978). "Lown-Ganong-Levine syndrome". Circulation. Ovid Technologies (Wolters Kluwer Health). 58 (3): 576–577. doi:10.1161/01.cir.58.3.576. ISSN 0009-7322. PMID 679452.
  3. DOUGLAS, JOHN E. (1972). "Lown-Ganong-Levine Syndrome". Circulation. Ovid Technologies (Wolters Kluwer Health). 45 (5): 1143–1144. doi:10.1161/01.cir.45.5.1143. ISSN 0009-7322. PMID 5020803.
  4. 4.0 4.1 Benditt, D G; Pritchett, L C; Smith, W M; Wallace, A G; Gallagher, J J (1978). "Characteristics of atrioventricular conduction and the spectrum of arrhythmias in lown-ganong-levine syndrome". Circulation. Ovid Technologies (Wolters Kluwer Health). 57 (3): 454–465. doi:10.1161/01.cir.57.3.454. ISSN 0009-7322. PMID 624155.
  5. Denes, Pablo; Wu, Delon; Rosen, Kenneth M. (1974). "Demonstration of Dual A-V Pathways in a Patient with Lown-Ganong-Levine Syndrome". Chest. Elsevier BV. 65 (3): 343–346. doi:10.1378/chest.65.3.343. ISSN 0012-3692.
  6. Lankveld TA, Zeemering S, Crijns HJ, Schotten U (July 2014). "The ECG as a tool to determine atrial fibrillation complexity". Heart. 100 (14): 1077–84. doi:10.1136/heartjnl-2013-305149. PMID 24837984.
  7. Harris K, Edwards D, Mant J (2012). "How can we best detect atrial fibrillation?". J R Coll Physicians Edinb. 42 Suppl 18: 5–22. doi:10.4997/JRCPE.2012.S02. PMID 22518390.
  8. Cosío FG (June 2017). "Atrial Flutter, Typical and Atypical: A Review". Arrhythm Electrophysiol Rev. 6 (2): 55–62. doi:10.15420/aer.2017.5.2. PMC 5522718. PMID 28835836.
  9. Katritsis DG, Josephson ME (August 2016). "Classification, Electrophysiological Features and Therapy of Atrioventricular Nodal Reentrant Tachycardia". Arrhythm Electrophysiol Rev. 5 (2): 130–5. doi:10.15420/AER.2016.18.2. PMC 5013176. PMID 27617092.
  10. Letsas KP, Weber R, Siklody CH, Mihas CC, Stockinger J, Blum T, Kalusche D, Arentz T (April 2010). "Electrocardiographic differentiation of common type atrioventricular nodal reentrant tachycardia from atrioventricular reciprocating tachycardia via a concealed accessory pathway". Acta Cardiol. 65 (2): 171–6. doi:10.2143/AC.65.2.2047050. PMID 20458824.
  11. "Atrioventricular Nodal Reentry Tachycardia (AVNRT) - StatPearls - NCBI Bookshelf".
  12. Schernthaner C, Danmayr F, Strohmer B (2014). "Coexistence of atrioventricular nodal reentrant tachycardia with other forms of arrhythmias". Med Princ Pract. 23 (6): 543–50. doi:10.1159/000365418. PMC 5586929. PMID 25196716.
  13. Scher DL, Arsura EL (September 1989). "Multifocal atrial tachycardia: mechanisms, clinical correlates, and treatment". Am. Heart J. 118 (3): 574–80. doi:10.1016/0002-8703(89)90275-5. PMID 2570520.
  14. Goodacre S, Irons R (March 2002). "ABC of clinical electrocardiography: Atrial arrhythmias". BMJ. 324 (7337): 594–7. doi:10.1136/bmj.324.7337.594. PMC 1122515. PMID 11884328.
  15. Lin CY, Lin YJ, Chen YY, Chang SL, Lo LW, Chao TF, Chung FP, Hu YF, Chong E, Cheng HM, Tuan TC, Liao JN, Chiou CW, Huang JL, Chen SA (August 2015). "Prognostic Significance of Premature Atrial Complexes Burden in Prediction of Long-Term Outcome". J Am Heart Assoc. 4 (9): e002192. doi:10.1161/JAHA.115.002192. PMC 4599506. PMID 26316525.
  16. Strasburger JF, Cheulkar B, Wichman HJ (December 2007). "Perinatal arrhythmias: diagnosis and management". Clin Perinatol. 34 (4): 627–52, vii–viii. doi:10.1016/j.clp.2007.10.002. PMC 3310372. PMID 18063110.
  17. Rao AL, Salerno JC, Asif IM, Drezner JA (July 2014). "Evaluation and management of wolff-Parkinson-white in athletes". Sports Health. 6 (4): 326–32. doi:10.1177/1941738113509059. PMC 4065555. PMID 24982705.
  18. Rosner MH, Brady WJ, Kefer MP, Martin ML (November 1999). "Electrocardiography in the patient with the Wolff-Parkinson-White syndrome: diagnostic and initial therapeutic issues". Am J Emerg Med. 17 (7): 705–14. doi:10.1016/s0735-6757(99)90167-5. PMID 10597097.
  19. Glinge C, Sattler S, Jabbari R, Tfelt-Hansen J (September 2016). "Epidemiology and genetics of ventricular fibrillation during acute myocardial infarction". J Geriatr Cardiol. 13 (9): 789–797. doi:10.11909/j.issn.1671-5411.2016.09.006. PMC 5122505. PMID 27899944.
  20. Samie FH, Jalife J (May 2001). "Mechanisms underlying ventricular tachycardia and its transition to ventricular fibrillation in the structurally normal heart". Cardiovasc. Res. 50 (2): 242–50. doi:10.1016/s0008-6363(00)00289-3. PMID 11334828.
  21. Adabag AS, Luepker RV, Roger VL, Gersh BJ (April 2010). "Sudden cardiac death: epidemiology and risk factors". Nat Rev Cardiol. 7 (4): 216–25. doi:10.1038/nrcardio.2010.3. PMC 5014372. PMID 20142817.
  22. Koplan BA, Stevenson WG (March 2009). "Ventricular tachycardia and sudden cardiac death". Mayo Clin. Proc. 84 (3): 289–97. doi:10.1016/S0025-6196(11)61149-X. PMC 2664600. PMID 19252119.
  23. Levis JT (2011). "ECG Diagnosis: Monomorphic Ventricular Tachycardia". Perm J. 15 (1): 65. doi:10.7812/tpp/10-130. PMC 3048638. PMID 21505622.
  24. LOWN B, GANONG WF, LEVINE SA (1952). "The syndrome of short P-R interval, normal QRS complex and paroxysmal rapid heart action". Circulation. 5 (5): 693–706. doi:10.1161/01.cir.5.5.693. PMID 14926053.
  25. Benditt, D G; Klein, G J; Kriett, J M; Dunnigan, A; Benson, D W (1984). "Enhanced atrioventricular nodal conduction in man: electrophysiologic effects of pharmacologic autonomic blockade". Circulation. Ovid Technologies (Wolters Kluwer Health). 69 (6): 1088–1095. doi:10.1161/01.cir.69.6.1088. ISSN 0009-7322. PMID 6713613.
  26. Caracta, Anthony R.; Damato, Anthony N.; Gallagher, John J.; Josephson, Mark E.; Varghese, P.Jacob; Lau, Sun H.; Westura, Edwin E. (1973). "Electrophysiologic studies in the syndrome of short P-R interval, normal QRS complex". The American Journal of Cardiology. Elsevier BV. 31 (2): 245–253. doi:10.1016/0002-9149(73)91037-0. ISSN 0002-9149.