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Lown-Ganong-Levine Syndrome

Atrial Tachyarrhythmia with Short PR Interval

Lown-Ganong-Levine syndrome is part of the preexcitation syndromes cluster, a condition with no definite structural support, manifesting as tachycardia paroxysms and a typical electrocardiogram aspect, with short PR interval, normal QRS complex duration and absent delta wave.


Presentation

Lown-Ganong-Levine syndrome (LGLS) patients usually have no cardiovascular symptoms between tachycardia crisis. The ailment most often affects men [1] in their second or third decade of life [2], but signs may occur at any age and consist of rapid, regular palpitations caused by a heart rate of 200 beats per minute or more. Episodes typically begin and end suddenly, but the latter may also be perceived as gradual because the episode is followed by sinus tachycardia. Diminished cardiac output caused by the fact that diastole duration is diminished leads to lightheadedness and dyspnea. Chest pain is encountered in victims with coronary artery disease (due to the fact that coronary supply is ensured during diastole), hypertrophic cardiomyopathy or structural heart malformations, such as tricuspid atresia, Ebstein’s anomaly, mitral valve prolapse or corrected transposition of great vessels. Atrial fibrillation may be experienced in such cases, but usually, have short duration [3]. During the attack, arterial hypotension may be recorded. In advanced situations, syncope is noticed by ventricular tachycardia or ventricular fibrillation. Patients sometimes present with heart failure symptoms. The episodes may be followed by polyuria.

The frequency of the crisis tends to diminish with the advancement of age. The death risk is low, however, incidences have been recorded [4]. The demise mechanism is arrhythmic and always appears in patients that have experienced arrhythmia episodes before, so sudden death is never the first symptom [5].

Those who perceive palpitations in the cervical area are more likely to have nodal reentrant tachycardia instead of LGLS [6]. Vagal maneuvers, such as Valsalva or carotid sinus massage may block atrioventricular node conduction and stop the arrhythmia if its technique involves the node, may have no effect or temporarily slow atrioventricular conduction. This clinical response is valuable while trying to establish the process of an paroxysmal tachycardia attack [7].

Tachycardia
  • Sixty-five patients with dual pathway atrioventricular (A-V) nodal reentrant paroxysmal tachycardia were studied.[ncbi.nlm.nih.gov]
  • The only morbidity associated with the syndrome is the occurrence of paroxysmal episodes of tachycardia which may be of several types, including sinus tachycardia, supraventricular tachycardia, atrial fibrillation, atrial flutter, or even ventricular[en.wikipedia.org]
  • Lown-Ganong-Levine syndrome (LGLS) patients usually have no cardiovascular symptoms between tachycardia crisis.[symptoma.com]
  • In three patients the atrium did not appear necessary to sustain supraventricular tachycardia.[ncbi.nlm.nih.gov]
  • 6/12 (50%), (group II) atrial fibrillation (AF) 2/12 (17%), group III) ventricular tachycardia (VT) 4/12 (33%).[ncbi.nlm.nih.gov]
Heart Disease
  • Lown Ganong Levine syndrome Symptoms Here are the list of Lown Ganong Levine syndrome Symptoms: palpitations lightheadedness shortness of breath heart disease or coronary artery disease cardiac stress chest pain[diseasesatoz.com]
  • Heart Disease Forum This expert forum is not accepting new questions. Please post your question in one of our medical support communities .[medhelp.org]
  • disease MANAGEMENT vagal manoeuvre’s avoid alcohol avoid coffee electrophysiological study and ablation EPONYMS Bernard Lown William Francis Ganong Jr Samuel Albert Levine About Chris Nickson FCICM FACEM BSc(Hons) BHB MBChB MClinEpid(ClinTox) DipPaeds[lifeinthefastlane.com]

Workup

LGLS diagnosis is mainly based on electrocardiography (ECG). The criteria include a normal or inverted P wave, a short PR interval less than or equal to 0.12 second (120 ms), with normal QRS complex duration, the absence of a delta wave and presence of clinical complaints consisting of tachycardia episodes. However, QRS complexes may also be wide, if the sufferer has a preexisting or functional right bundle or left bundle branch block. A short PR interval may be seen in ectopic atrial rhythms and atrioventricular junctional rhythms [8]. When a patient describes typical supraventricular arrhythmia but normal sinus rhythm is observed on the electrocardiogram, Holter monitorization or implantable loop recorder are indicated. A Holter monitor is useful when symptoms are observed every day, whereas the other method is employed when crisis appear not as often. Obtaining an electrocardiography documentation is particularly important because it can offer important information about the mechanism of the tachycardia.

Blood tests should include serum thyroid-stimulating hormone electrolytes, calcium, and magnesium level. Further information is provided by vagal maneuvers. A carotid sinus massage can only be performed if the subject has no history of cerebrovascular events, has no carotid bruits and the blood pressure is not decreased. It should be done under blood pressure and ECG monitorization. This maneuver can terminate the episode or induce transient atrioventricular block.

A novel technique is represented by noninvasive cardiac mapping using computed-tomography scan–based three-dimensional electroimaging and 252-lead ECG [9]. When the condition becomes intolerable or when a ventricular arrhythmia is suspected, an invasive electrophysiology study is needed.

Short PR Interval
  • Levine . [1] [2] Individuals with a short PR interval found incidentally on EKG were once thought to have LGL syndrome.[en.wikipedia.org]
  • Therefore, neither the short PR interval nor the narrow QRS complexes characterized these forms of pre-excitation.[ncbi.nlm.nih.gov]
  • A short PR interval may be seen in ectopic atrial rhythms and atrioventricular junctional rhythms.[symptoma.com]
  • His electrocardiogram showed a short PR interval and a normal QRS complex after abortion of supraventricular tachycardia in favor of Lown-Ganong-Levine syndrome or enhanced atrioventricular nodal conduction.[ncbi.nlm.nih.gov]
  • Causes of short PR interval: Lown-Ganong-Levine Syndrome. Wolff-Parkinson-White (WPW) Syndrome: Short PR interval, normal P wave, delta wave resulting in a wide QRS complex.[metealpaslan.com]

Treatment

  • WPW Syndrome ECG Treatment of Wolff-Parkinson-White syndrome : Asymptomatic patients, showing only ECG changes do not require treatment.[doctortipster.com]
  • Lown-Ganong-Levine syndrome requires treatment only if the rapid heart rate causes symptoms, or an abnormal heart rhythm occurs.[freemd.com]
  • This service should be used for second opinions, additional medical reviews and consultations; not for primary diagnosis or treatment.[xmri.com]
  • There is no treatment for it unless the tachycardia is affecting their life style. EKG is the way to diagnose it. If they had attacks of syncope, they should not drive.[answers.yahoo.com]

Prognosis

  • Prognosis [ edit ] Individuals with LGL syndrome do not carry an increased risk of sudden death.[en.wikipedia.org]
  • Prognosis Individuals with LGL syndrome do not carry an increased risk of sudden death.[ipfs.io]
  • Prognosis The syndrome can produce ventricular fibrillation and sudden death. [ 5 ] However, it is normally far more benign and can usually be controlled by pharmacological means.[patient.info]
  • Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis.[doi.org]
  • The prognosis may be bleak when both circumstances manifest in the same individual. Evaluation of an altered PR-interval always should be made before hospital discharge. 1. Eshtehardi P, Koestner SC, Adorjan P, et al. (2009).[webmedcentral.com]

Etiology

  • Therefore, most consider the disorder to be a clinical syndrome with multiple different underlying etiologies, all involving some form of intranodal or paranodal fibers that bypass all or part of the atrioventricular (AV) node with subsequent conduction[ipfs.io]
  • Takotsubo disease is being increasingly studied, but we do not know still its fundamental etiology. Its occurrence is not uncommon in individuals with previous cardiac electrical disorders.[webmedcentral.com]
  • ETIOLOG A Formas Adquiridas. Formas Cong nitas: - Heredables. - No Heredables.[ilustrados.com]

Epidemiology

  • Young JB; American Heart Association; Council on Clinical Cardiology, Heart Failure, and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; Council on Epidemiology[doi.org]
  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
  • Feeds For International Users Institutions/Librarians FAQ Subscriber Help All Issues January 1, 1950 - November 20, 2018 Subjects All Subjects Arrhythmia and Electrophysiology Basic, Translational, and Clinical Research Critical Care and Resuscitation Epidemiology[circ.ahajournals.org]
  • Epidemiology Lown suggested that 17% of people with a PR interval of less than 120 ms would have the condition. [ 2 ] Presentation History The history is of bouts of tachycardia that may present as rapid palpitations.[patient.info]
  • Epidemiology Frequency United StatesLown and associates described tachyarrhythmias in 17% of patients with a short PR interval. [3] Some 2-4% of the adult population has a PR interval less than or equal to 0.12 second. [23] Taken together, these data[thehealthscience.com]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber).[ncbi.nlm.nih.gov]
  • Pathophysiology [ edit ] Unlike WPW syndrome , the pathophysiology underlying LGL syndrome is poorly understood. The syndrome was once thought to involve an accessory pathway (bundle of James) that connects the atria directly to the bundle of His.[en.wikipedia.org]
  • Pathophysiology Unlike WPW syndrome , the pathophysiology underlying LGL syndrome is poorly understood. The syndrome was once thought to involve an accessory pathway (bundle of James) that connects the atria directly to the bundle of His.[ipfs.io]
  • Levine . [ 1 ] [ 2 ] A short PR is seen. [ 3 ] Pathophysiology In normal individuals, electrical activity in the heart is initiated in the sinoatrial (SA) node (located in the right atrium ), propagates to the atrioventricular (AV) node, and then through[dictionnaire.sensagent.leparisien.fr]
  • Pathophysiology No single structural anomaly has been implicated directly as the cause of LGL. Indeed, most authors believe that LGL does not exist as a phenomenon separate from other known conditions.[odlarmed.com]

Prevention

  • Statement from the Council on Clinical Cardiology, Heart Failure, and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention[doi.org]
  • Institutions/Librarians FAQ Subscriber Help All Issues January 1, 1950 - November 20, 2018 Subjects All Subjects Arrhythmia and Electrophysiology Basic, Translational, and Clinical Research Critical Care and Resuscitation Epidemiology, Lifestyle, and Prevention[circ.ahajournals.org]
  • Arrhythmic chronic treatment, designed to prevent crisis of ventricular tachycardia and to control ventricular rate in case of atrial fibrillation is realized by the administration of class IC antyarrhythmics (flecainide, propafenone), class III antiarrhythmics[doctortipster.com]
  • Bernard Lown was a founder of International Physicians for the Prevention of Nuclear War and, in 1985, he was awarded the Nobel Prize for Peace. He is Professor Emeritus of Cardiology at Harvard.[patient.info]
  • Although disappointed by the inability to ablate the tachycardia, the decision was made to begin a home medication regimen to help with prevention and breakthrough. Propranolol 10 mg by mouth daily was prescribed for initial treatment.[eplabdigest.com]

References

Article

  1. Rodriguez LM, De Chillou C, Schläpfer J, et al. Age at onset and gender of patients with different types of supraventricular tachycardias. Am J Cardiol. 1992;70:1213-5.
  2. Tada H, Oral H, Greenstein R, et al. Analysis of age of onset of accessory pathway-mediated tachycardia in men and women. Am J Cardiol. 2002;89:470-1.
  3. Sharma MK, Misra S. Anaesthetic management of a patient with Lown-Ganong-Levine syndrome – a case report. Med J Armed Forces India. 2011;67(3):285-7.
  4. Santinelli V, Radinovic A, Manguso F, et al. Asymptomatic ventricular preexcitation: a long-term prospective follow-up study of 293 adult patients. Circ Arrhythm Electrophysiol. 2009;2:102-7.
  5. Santinelli V, Radinovic A, Manguso F, et al. The natural history of asymptomatic ventricular pre-excitation a long-term prospective follow-up study of 184 asymptomatic children. J Am Coll Cardiol. 2009;53:275-80.
  6. González-Torrecilla E, Almendral J, Arenal A, et al. Combined evaluation of bedside clinical variables and the electrocardiogram for the differential diagnosis of paroxysmal atrioventricular reciprocating tachycardias in patients without pre-excitation. J Am Coll Cardiol. 2009;53:2353-8.
  7. Marchlinski FE. Diagnosing the mechanism of supraventricular tachycardia: restoring the luster of a fading art. J Am Coll Cardiol. 2009;53:2359-61.
  8. Mackenzie R. Short PR interval. J Insur Med. 2005;37:145–152.
  9. Shah A, Hocini M, Haissaguerre M, Jaïs P. Non-invasive mapping of cardiac arrhythmias. Curr Cardiol Rep. 2015;17(8):60.

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Last updated: 2018-06-21 20:34