Lown-Ganong-Levine syndrome: Difference between revisions

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{{Infobox_Disease |
  Name          = {{PAGENAME}} |
  Image          = |
  Caption        = |
  DiseasesDB    = 7599 |
  ICD10          = {{ICD10|I|45|6|i|30}}  |
  ICD9          = {{ICD9|426.81}} |
  ICDO          = |
  OMIM          = 108950 |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D008151 |
}}
{{SI}}
{{CMG}} '''Associate Editor-In-Chief:'''
__NOTOC__
__NOTOC__
{{SI}}
{{SI}}                                                                 
{{CMG}}
==Overview==
==Overview==
'''Lown-Ganong-Levine syndrome''' ('''LGL''') is a syndrome of pre-excitation of the [[Ventricle (heart)|ventricles]] due to an accessory pathway providing an abnormal electrical communication from the [[atrium (anatomy)|atria]] to the ventricles. It is grouped with [[Wolff-Parkinson-White syndrome]] as an atrioventricular re-entry tachycardia (AVRT). The syndrome is [[list of eponymous diseases|named after]] [[Bernard Lown]], William Francis Ganong, Jr., and Samuel A. Levine.<ref name="pmid14926053">{{cite journal| author=LOWN B, GANONG WF, LEVINE SA| title=The syndrome of short P-R interval, normal QRS complex and paroxysmal rapid heart action. | journal=Circulation | year= 1952 | volume= 5 | issue= 5 | pages= 693-706 | pmid=14926053 | doi= | pmc= | url= }} </ref>


==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
==Classification==
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
:*[group1]
:*[group2]
:*[group3]
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
==Clinical Features== 


In normal individuals, electrical activity in the heart is initiated in the [[sinoatrial node|sinoatrial]] (SA) node (located in the [[right atrium]]), propagates to the [[atrioventricular node| atrioventricular]] (AV) node, and then through the [[bundle of His]] to the ventricles of the heart. (See [[electrical conduction system of the heart]]).
==Differentiating [disease name] from other Diseases==
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
===Age===
*Patients of all age groups may develop [disease name].
*[Disease name] is more commonly observed among patients aged [age range] years old.
*[Disease name] is more commonly observed among [elderly patients/young patients/children].
===Gender===
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
===Race===
*There is no racial predilection for [disease name].
*[Disease name] usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop [disease name].


The AV node acts as a gatekeeper, limiting the electrical activity that reaches the ventricles of the heart.  This is an important function of the AV node, because if the signals generated in the atria of the heart were to increase in rate (such as during [[atrial fibrillation]] or [[atrial flutter]]), the AV node will limit the electrical activity that conducts to the ventricles.  For instance, if the atria are electrically activated at 300 beats per minute, half those electrical impulses are blocked by the AV node, so that the ventricles are activated at 150 beats per minute (giving a [[pulse]] of 150 beats per minute).  Another important property of the AV node is that it slows down individual electrical impulses.  This is manifest on the [[electrocardiogram|EKG]] as the PR interval, the time from activation of the atria (manifest as the P wave) and activation of the ventricles (manifest as the QRS complex).
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].


Individuals with LGL syndrome are thought to have an accessory pathway that connects the atria directly to the bundle of His. As in WPW syndrome, the accessory pathway does not share the rate-slowing properties of the AV node, and may conduct electrical activity at a significantly higher rate than the AV node. For instance, in the example above, if an individual had an atrial rate of 300 beats per minute, the accessory bundle may conduct all the electrical impulses from the atria to the ventricles, causing the ventricles to activate at 300 beats per minute. Because the ventricles are the main pumping chambers of the heart, the body depends on the proper filling and emptying (contraction) of them.  When conducted too rapidly (i.e. 300 beats per minute), the body would become hemodynamically unstable.  If not corrected quickly, the ventricles can fibrillate causing VF (ventricular fibrillation)- leading to sudden cardiac death (SCD).
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].  
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].


==Characteristic Findings==
== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]
=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]


* [[PR interval]] is shorter than normal,<ref name="pmid14662232">{{cite journal| author=Eichholz A, Whiting RB, Artal R| title=Lown-Ganong-Levine syndrome in pregnancy. | journal=Obstet Gynecol | year= 2003 | volume= 102 | issue= 6 | pages= 1393-5 | pmid=14662232 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14662232  }} </ref> but no delta wave is present(in contrast to [[Wolff Parkinson White syndrome]] where there is a delta wave which is a slurring of the [[QRS]] upstroke)
=== Laboratory Findings ===
* LGL is due to intranodal bypass tracts (i.e. there is conduction down James fibers) 
*There are no specific laboratory findings associated with [disease name].
* [[QRS duration]] is normal
* [[PR interval]] is less than 0.12 seconds
* [[P wave]] is normal


==Diagnosis==
*A  [positive/negative] [test name] is diagnostic of [disease name].
LGL syndrome is commonly diagnosed on the basis of the surface [[electrocardiogram|EKG]] in an [[asymptomatic]] individual. In this case it is manifest as a PR interval less than or equal to 0.12 second (120 ms) with normal QRS complex duration.
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].


It can be distinguished from WPW syndrome because:
== Treatment ==
* The QRS complexes in LGL syndrome are normal because ventricular contraction is initiated in the normal manner. The broad complexes seen in the asymptomatic individual with WPW are not a feature of LGL.
=== Medical Therapy ===
* The delta waves seen in WPW syndrome are not seen in LGL syndrome as the accessory pathway does not connect to the ventricles and so ventricular contraction does not start early.
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].


==Differential Diagnosis==
*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
*[[Paroxysmal Atrial Tachycardia (PAT)]]
*[[Wolf-Parkinson-White syndrome|Wolf Parkinson White Syndrome]]
*[[Mahaim Type Preexcitation]]


==Related Chapters==
* [[Wolff-Parkinson-White syndrome]]
* [[Cardiac electrophysiology]]
* [[Electrical conduction system of the heart]]
* [[Electrocardiogram]]
* [[Electrophysiologic study]]
==References==
==References==
{{reflist|2}}
{{Reflist|2}}
{{Electrocardiography}}
{{Circulatory system pathology}}
[[Category:Pick One of 28 Approved]]


[[Category:Electrophysiology]]
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[[Category:Cardiology]]
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[[Category:Arrhythmia]]
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Revision as of 04:35, 9 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Clinical Features

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action 1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

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