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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].

Coarctation of the Aorta
  • He had previously undergone balloon dilatation for coarctation of the aorta. Pediatric cardiologist Dr. Mahmut Gokdemir has donated the above ECG to our website. Click here for a more detailed ECG ECG 4a.[metealpaslan.com]
Tachycardia
  • The most common tachycardias include AV nodal reentry, accessory pathway mediated tachycardia, atrial fibrillation, atrial flutter, and ventricular tachycardia. [23, 26] To date, the underlying mechanisms that generate a short PR interval in LGL have[emedicine.com]
  • Abstract 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]
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]
  • Lown Ganong Levine syndrome Synonym for Pre-excitation syndrome I00-I99 Diseases of the circulatory system (I00-I99) I30-I52 Other forms of heart disease (I30-I52) I45 Other conduction disorders Results for Lown Ganong Levine syndrome and additional synonyms[averbis.com]
  • […] palpitations collapse INVESTIGATIONS ECG: - very short PR intervals - sinus rhythm with ventricular extrasystoles - no delta wave (how to differentiate from WPW) - normal QRS Holter monitor: - frequency and severity of attacks ECHO: to rule out structural heart[litfl.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]
  • Heart Disease Forum This expert forum is not accepting new questions. Please post your question in one of our medical support communities.[medhelp.org]
Carotid Bruit
  • 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.[symptoma.com]
Suggestibility
  • Frequency distribution analyses of P-R and A-H intervals in the 65 patients demonstrated continuous unimodal functions, suggesting a continuum of A-V nodal properties.[ncbi.nlm.nih.gov]
  • Theories to explain the condition have suggested possible accessory fibres that bypass all, or part of, the AV node [ 1 ] . In some cases there may be a congenitally malfunctioning AV node [ 2 ] .[patient.co.uk]
  • These findings suggest that preferential rapidly conducting A-V nodal fibers and intranodal reentry are the responsible mechanisms in those patients with Lown-Ganong-Levine syndrome and reciprocating tachycardia.[ncbi.nlm.nih.gov]
  • We suggest that enhanced A-V nodal conduction in this patient was due to the developmental defect in the A-V node; this abnormality caused a loss of specific impulse-delaying function, and thus allowed rapid, unfiltered atrial impulses to reach the lower[ncbi.nlm.nih.gov]
  • These findings suggested that preferential rapidly conducting A-V nodal and intranodal reentry are the responsible mechanisms in this reciprocating tachycardia.[ncbi.nlm.nih.gov]
Forgetful
  • Aside from the occasional palpitations that I experience (usually when I forget to take my medication), I am symptom free.[sumonova.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.

Left Axis Deviation
  • axis deviation ) · QT ( Short QT syndrome ) · T ( T wave alternans ) · ST ( Osborn wave, ST elevation, ST depression ) Cardiomegaly Ventricular hypertrophy ( Left, Right / Cor pulmonale ) · Atrial enlargement ( Left, Right ) Other Cardiac fibrosis ·[dictionnaire.sensagent.leparisien.fr]
Narrow QRS Complex
  • Therefore, neither the short PR interval nor the narrow QRS complexes characterized these forms of pre-excitation.[ncbi.nlm.nih.gov]
  • The patient had a history of recurrent attacks of tachyarrhythmia and his electrocardiogram showed a short P-R interval (0.10 sec) with narrow QRS complex and left ventricular hypertrophy with giant negative T waves.[ncbi.nlm.nih.gov]
  • QRS complex ( 120 msec); regular, wide QRS complex ( 120 msec); may not see any P-wave activity in either type (atypical or typical) Atypical AVNRT: RP interval PR interval; P waves negative in leads III and aVF Typical AVNRT: RP interval PR interval[explainmedicine.com]
Short PR Interval
  • However, none of the identified short PR interval mechanisms is necessary for the generation of LGL tachycardias.[emedicine.com]
  • Therefore, neither the short PR interval nor the narrow QRS complexes characterized these forms of pre-excitation.[ncbi.nlm.nih.gov]
  • From Wikidata Jump to navigation Jump to search syndrome that involves pre-excitation of the ventricles due to an accessory pathway providing an abnormal electrical communication from the atria to the ventricles atrial tachyarrhythmia with short PR interval[wikidata.org]
  • Levine. [1] [2] Individuals with a short PR interval found incidentally on EKG were once thought to have LGL syndrome.[en.wikipedia.org]

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]
  • Click here for example ECG and further information Links: treatment prophylaxis[gpnotebook.co.uk]
  • However, verapamil and digoxin are contraindicated for treatment of atrial fibrillation or flutter in these patients because they might accelerate conduction through the bypass tract and induce ventricular fibrillation.[accessanesthesiology.mhmedical.com]

Prognosis

  • Prognosis [ edit ] Individuals with LGL syndrome do not carry an increased risk of sudden death.[en.wikipedia.org]
  • Prognosis The syndrome can produce ventricular fibrillation and sudden death [ 7 ] . However, this is unusual, and it is normally far more benign and can be controlled by pharmacological means.[patient.co.uk]
  • Prognosis Individuals with LGL syndrome do not carry an increased risk of sudden death.[ipfs.io]
  • 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]
  • Prognosis The syndrome can produce ventricular fibrillation and sudden death [ 7 ]. However, this is unusual, and it is normally far more benign and can be controlled by pharmacological means.[patient.info]

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]

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]
  • Epidemiology Lown suggested that 17% of people with a PR interval of less than 120 ms would have the condition [ 3 ] . It is however very rare, with prevalence estimated to be less than one in a million [ 4 ] .[patient.co.uk]
  • Epidemiology Lown suggested that 17% of people with a PR interval of less than 120 ms would have the condition [ 3 ]. It is however very rare, with prevalence estimated to be less than one in a million [ 4 ].[patient.info]
  • “Lown-Ganong-Levine Syndrome: Background, Pathophysiology, Epidemiology.” Medscape. Web. 5 Nov. 2015. MacKenzie, MD, Ross. “Short PR Interval.” Journal of Insurance Medicine 37 (2005): 145-52. Web. 5 Nov. 2015. Lown B, Ganong WF, Levine SA.[emdocs.net]
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]
  • Observations regarding the pathophysiology of the Lown-Ganong-Levine syndrome. Am J Cardiol. 1982; 49 (2):283–90. [ PubMed ] [ Google Scholar ] 6. Dougherty A, Naccarelli G.[ncbi.nlm.nih.gov]
  • Whatever the pathophysiology, conduction is passed from the atria to the bundle of His without the delay usually incurred at the AV node.[patient.co.uk]

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]
  • Bernard Lown was a founder of International Physicians for the Prevention of Nuclear War and, in 1985, the group was awarded the Nobel Prize for Peace. He developed the direct current defibrillator.[patient.co.uk]
  • Hypoxia, hypercarbia, or acidosis must be prevented because all these complications render cardiac muscle membranes unstable and ectopic depolarization more likely. Same as for Wolff-Parkinson-White syndrome.[accessanesthesiology.mhmedical.com]
  • 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]
  • The key to successful management is in avoiding precipitating factors, vigilant pre-operative monitoring of dysrhythmias, and prevention and aggressive management of complications on occurrence.[ncbi.nlm.nih.gov]

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