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Lown-Ganong-Levine syndrome

MedGen UID:
354734
•Concept ID:
C1862387
•
Disease or Syndrome
Synonym: Atrial tachyarrhythmia with short pr interval
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Autosomal dominant inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0007174
OMIM®: 108950
Orphanet: ORPHA844

Definition

Lown-Ganong-Levine syndrome is an extremely rare conduction disorder characterized by a short PR interval (less than or equal to 120 ms) with normal QRS complex on electrocardiogram associated with the occurrence of episodes of atrial tachyarrythmias (e.g. atrial fibrillation, atrial tachycardia). [from ORDO]

Clinical features

From HPO
Paroxysmal atrial tachycardia
MedGen UID:
10594
•Concept ID:
C0030587
•
Disease or Syndrome
A disorder characterized by an electrocardiographic finding of episodic atrial tachycardia with abrupt onset and termination.
See: Feature record | Search on this feature
Paroxysmal atrial fibrillation
MedGen UID:
115990
•Concept ID:
C0235480
•
Disease or Syndrome
Episodes of atrial fibrillation that typically last for several hours up to one day and terminate spontaneously.
See: Feature record | Search on this feature
Shortened PR interval
MedGen UID:
105466
•Concept ID:
C0520878
•
Finding
Reduced time for the PR interval (beginning of the P wave to the beginning of the QRS complex). In adults, normal values are 120 to 200 ms long.
See: Feature record | Search on this feature
Permanent atrial fibrillation
MedGen UID:
750308
•Concept ID:
C2586056
•
Pathologic Function
Atrial fibrillation (AF) that cannot be successfully terminated by cardioversion, and longstanding (more than 1 year) AF, where cardioversion is not indicated or has not been attempted, is termed permanent.
See: Feature record | Search on this feature

Professional guidelines

PubMed

Treatment of drug resistant A-V reciprocating tachycardias with multiprogrammable dual demand A-V sequential (DVI,MN) pacemakers.
Portillo B, Medina-Ravell V, Portillo-Leon N, Maduro C, Mejias J, Berkovits BV, Castellanos A
Pacing Clin Electrophysiol 1982 Nov;5(6):814-25. doi: 10.1111/j.1540-8159.1982.tb06562.x. PMID: 6184682

Recent clinical studies

Etiology

Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death.
Basso C, Corrado D, Rossi L, Thiene G
Circulation 2001 Jan 16;103(2):269-75. doi: 10.1161/01.cir.103.2.269. PMID: 11208688
High incidence of pre-excitation syndrome in Japanese families with Leber's hereditary optic neuropathy.
Mashima Y, Kigasawa K, Hasegawa H, Tani M, Oguchi Y
Clin Genet 1996 Dec;50(6):535-7. doi: 10.1111/j.1399-0004.1996.tb02732.x. PMID: 9147893
The preexcitation syndrome: epidemiological and genetic study.
Duraković Z, Duraković A, Kastelan A
Int J Cardiol 1992 May;35(2):181-6. doi: 10.1016/0167-5273(92)90175-3. PMID: 1572738
Atrial depolarization in Wolf-Parkinson-White and Lown-Ganong-Levine syndrome: vectorcardiographic features.
Zoneraich O, Zoneraich S
Chest 1979 Jul;76(1):70-5. doi: 10.1378/chest.76.1.70. PMID: 156108
Characteristics of atrioventricular conduction and the spectrum of arrhythmias in lown-ganong-levine syndrome.
Benditt DG, Pritchett LC, Smith WM, Wallace AG, Gallagher JJ
Circulation 1978 Mar;57(3):454-65. doi: 10.1161/01.cir.57.3.454. PMID: 624155

Diagnosis

Lown-Ganong-Levine syndrome in pregnancy.
Eichholz A, Whiting RB, Artal R
Obstet Gynecol 2003 Dec;102(6):1393-5. doi: 10.1016/s0029-7844(03)00708-7. PMID: 14662232
Lown-Ganong-Levine syndrome.
Catalano JT
Crit Care Nurse 1988 Jul-Aug;8(5):74-7. PMID: 3269321
The Lown-Ganong-Levine syndrome.
Castellanos A, Zaman L, Moleiro F, Aranda JM, Myerburg RJ
Pacing Clin Electrophysiol 1982 Sep;5(5):715-40. doi: 10.1111/j.1540-8159.1982.tb02310.x. PMID: 6182544
Atrial depolarization in Wolf-Parkinson-White and Lown-Ganong-Levine syndrome: vectorcardiographic features.
Zoneraich O, Zoneraich S
Chest 1979 Jul;76(1):70-5. doi: 10.1378/chest.76.1.70. PMID: 156108
Taboparesis: Lown-Ganong-Levine syndrome.
Garrett C
Proc R Soc Med 1972 Jun;65(6):563-4. PMID: 5035914Free PMC Article

Therapy

Clinical and evolutive aspects in ventricular preexcitation syndromes in child.
Dimitriu AG, Iordache C, Nicolau I, Anton-Goţia G, Frasin M, Nistor N, Varlam L, Streangă V, Jităreanu C
Rev Med Chir Soc Med Nat Iasi 1996 Jul-Dec;100(3-4):95-8. PMID: 9455443
Enhanced A-V nodal conduction (Lown-Ganong-Levine syndrome) by congenitally hypoplastic A-V node.
Ometto R, Thiene G, Corrado D, Vincenzi M, Rossi L
Eur Heart J 1992 Nov;13(11):1579-84. doi: 10.1093/oxfordjournals.eurheartj.a060104. PMID: 1464347
Diagnosis and management of the preexcitation syndromes.
Prystowsky EN
Curr Probl Cardiol 1988 Apr;13(4):225-310. doi: 10.1016/0146-2806(88)90025-4. PMID: 3292155
Mechanisms of junctional tachycardias in the Lown-Ganong-Levine syndrome.
Ward DE, Camm J
Am Heart J 1983 Jan;105(1):169-75. doi: 10.1016/0002-8703(83)90304-6. PMID: 6849236
How to spot...and care for...the patient with Lown-Ganong-Levine syndrome.
Skorga P, Nunnery C
Nursing 1981 Mar;11(3):37-42. doi: 10.1097/00152193-198103000-00003. PMID: 6907768

Prognosis

Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death.
Basso C, Corrado D, Rossi L, Thiene G
Circulation 2001 Jan 16;103(2):269-75. doi: 10.1161/01.cir.103.2.269. PMID: 11208688
High incidence of pre-excitation syndrome in Japanese families with Leber's hereditary optic neuropathy.
Mashima Y, Kigasawa K, Hasegawa H, Tani M, Oguchi Y
Clin Genet 1996 Dec;50(6):535-7. doi: 10.1111/j.1399-0004.1996.tb02732.x. PMID: 9147893
Familial atrial tachyarrhythmia with short PR interval.
Brodsky M, Wu D, Denes P, Rosen KM
Arch Intern Med 1977 Feb;137(2):165-9. PMID: 836114

Clinical prediction guides

Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death.
Basso C, Corrado D, Rossi L, Thiene G
Circulation 2001 Jan 16;103(2):269-75. doi: 10.1161/01.cir.103.2.269. PMID: 11208688
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.
Bauernfeind RA, Swiryn S, Strasberg B, Palileo E, Wyndham C, Duffy CE, Rosen KM
Am J Cardiol 1982 Feb 1;49(2):283-90. doi: 10.1016/0002-9149(82)90502-1. PMID: 7058743
Response of atrial flutter to overdrive atrial pacing and intravenous disopyramide phosphate, singly and in combination.
Camm J, Ward D, Spurrell R
Br Heart J 1980 Sep;44(3):240-7. doi: 10.1136/hrt.44.3.240. PMID: 7426181Free PMC Article
Cycle length in atrioventricular nodal reentrant paroxysmal tachycardia with observations on the Lown-Ganong-Levine syndrome.
Bauernfeind RA, Ayres BF, Wyndham CC, Dhingra RC, Swiryn SP, Strasberg B, Rosen KM
Am J Cardiol 1980 Jun;45(6):1148-53. doi: 10.1016/0002-9149(80)90472-5. PMID: 7377112
Syndrome of short P-R, narrow QRS and repetitive supraventricular tachyarrhythmias: the possible occurrence of the R-on-T phenomenon and the limits of this syndrome.
Castellanos A, Vagueiro MC, Befeler B, Myerburg RJ
Eur J Cardiol 1975 Jan;2(3):337-42. PMID: 1149771

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