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].
Entire Body System
- Anemia
Pacemaker complication Pericarditis Myocarditis Valvular disease Psychiatric causes: Panic attack Anxiety Somatic Symptom Disorder Drugs and Medications: Alcohol Caffeine Drugs of abuse (e.g. cocaine) Medications (e.g. digoxin, theophylline) Tobacco Misc Anemia [wikem.org]
[…] associated with coronary heart disease, hyperthyroidism, diseases affecting cardiac muscle such as hypertrophic cardiomyopathy, diseases causing low blood oxygen such as asthma and emphysema; previous chest surgery; kidney disease; blood loss and pain; anemia [en.wikipedia.org]
Maxam-Gilbert method Maxam-Gilbert 法 May-Hegglin anomaly May-Hegglin 異常 McArdle disease McArdle 病 McCune-Albright syndrome McCune-Albright 症候群 Meckel diverticulum Meckel 憩室 Meckel syndrome Meckel 症候群 Medical Subject Headings 医学件名標目表 MeSH Mediterranean anemia [jpeds.or.jp]
- Weight Loss
Weight loss suggests hyperthyroidism. Palpitation can be precipitated by vomiting or diarrhea that leads to electrolyte disorders and hypovolemia. [en.wikipedia.org]
- Pathologist
It was first described by American pathologist John T Prior (1917-2007) at Syracuse College of Medicine, New York in 1964.[4] The incidence of LHIS increases with age. Characteristically, LHIS spares fossa ovalis and the septum looks dumbell shaped. [ijcva.com]
Gastrointestinal
- Diarrhea
Palpitation can be precipitated by vomiting or diarrhea that leads to electrolyte disorders and hypovolemia. [en.wikipedia.org]
Cardiovascular
- Tachycardia
Found on http://www.encyclo.co.uk/local/20973 Lown-Ganong-Levine syndrome (loun´ gan´ong lә-vīn´) an electrocardiographic abnormality consisting of a short PR interval and normal QRS complex associated with paroxysmal supraventricular tachycardia. [encyclo.co.uk]
Paroxysmal tachycardia. Arch. Int. Med. 27: 571, 1921. [johnsonfrancis.org]
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.medscape.com]
Lown-Ganong-Levine syndrome (LGLS) patients usually have no cardiovascular symptoms between tachycardia crisis. [symptoma.com]
- Heart Disease
Recommended low-density lipoprotein cholesterol target level in people with heart disease <100 mg/dl Lipid profile The greatest harm in the care of these patients is the frequent attribution of coronary artery or arteriosclerotic heart disease as the [medicaljoyworks.com]
We conclude that the abnormalities of signal conduction described in association with LGL syndrome is probably liable to be affected by some cardiac structural disease and interestingly we are reporting right side heart disease in such problem. [omicsonline.org]
disease MANAGEMENT vagal manoeuvre’s avoid alcohol avoid coffee electrophysiological study and ablation LITFL Lown–Ganong–Levine syndrome – ECG library Bernard Lown (1921 – ) – Eponymictionary William Francis ‘Fran’ Ganong Jr. (1924 – 2007) – Eponymictionary [litfl.com]
They are usually females, in middle life, devoid of organic heart disease and exhibit a snapping apical first heart sound. They do not demonstrate any of the features of anomalous A-V conduction. [doi.org]
- Hypotension
(Orthostatic hypotension) See also congenital (Q20-Q28, 745-747) [bionity.com]
This results in hypotension or other hemodynamic instability. If ventricular tachycardia or ventricular fibrillation occurs, syncope may occur. [medindia.net]
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. [symptoma.com]
( Orthostatic hypotension ) - Rheumatic fever See also congenital ( Q20-Q28, 745-747 ) [wikidoc.org]
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.
PR Interval
- Short PR Interval
Called also short PR syndrome. [encyclo.co.uk]
In the seminal paper Lown B et al observed that out of the 200 patients with short PR interval, 23 had paroxysmal tachycardia (11%), compared with 0.5 to 1% in a control group of 200 patients with normal PR interval. [johnsonfrancis.org]
Engelsk definition A form of ventricular pre-excitation characterized by a short PR interval and a normal QRS complex. [mesh.kib.ki.se]
However, none of the identified short PR interval mechanisms is necessary for the generation of LGL tachycardias. [emedicine.medscape.com]
Treatment
What Is the Treatment for Lown Ganong Levine Syndrome? Treatment of Lown-Ganong-Levine is presently focused on the utilization of antiarrhythmic drugs. [icliniq.com]
WPW Syndrome ECG Treatment of Wolff-Parkinson-White syndrome : Asymptomatic patients, showing only ECG changes do not require treatment. [doctortipster.com]
Treatment / Management Treatment of Lown-Ganong-Levine currently is centered around the use of antiarrhythmic medications and, when refractory, radiofrequency catheter ablation. [ncbi.nlm.nih.gov]
Prognosis
Sections Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Consultations Deterrence and Patient Education Pearls and Other Issues Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
Prognosis The overall prognosis of patients with LGL syndrome is good. [wikidoc.org]
Prognosis Individuals with LGL syndrome do not carry an increased risk of sudden death. [ipfs.io]
Lown-Ganong-Levine Syndrome In this article Epidemiology Presentation Investigations Management Prognosis Historical The Lown-Ganong-Levine (LGL) syndrome is one of the pre-excitation syndromes of which Wolff-Parkinson-White (WPW) syndrome is the best [patient.info]
Prognosis[edit] Individuals with LGL syndrome do not carry an increased risk of sudden death. [wikizero.com]
Etiology
Sections Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Consultations Deterrence and Patient Education Pearls and Other Issues Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
Etiology Some evidence suggests that LGL is a hereditary condition in some families. No definitive structural/anatomic anomaly has been identified as yet. [clinicalgate.com]
[…] of palpitations is long, and in some cases, the etiology is unable to be determined.[1] In one study reporting the etiology of palpitations, 43% were found to be of cardiac etiology, 31% of psychiatric etiology and approximately 10% were classified as [en.wikipedia.org]
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]
Tricuspid regurgitation is functional and is a satellite of left-sided heart disease and/or elevated pulmonary artery pressure most of the time; and when progressive, it worsens the patient prognosis whatever the underlying etiology [10]. [omicsonline.org]
Epidemiology
Sections Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Consultations Deterrence and Patient Education Pearls and Other Issues Enhancing Healthcare [pubmed.ncbi.nlm.nih.gov]
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 [wikidoc.org]
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]
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]
He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education. [lifeinthefastlane.com]
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 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]
Prevention
Prevention There are no primary preventive measures available for LGL syndrome. References ↑ 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). [wikidoc.org]
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]
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]
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]
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]
References
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Marchlinski FE. Diagnosing the mechanism of supraventricular tachycardia: restoring the luster of a fading art. J Am Coll Cardiol. 2009;53:2359-61.
- Mackenzie R. Short PR interval. J Insur Med. 2005;37:145–152.
- Shah A, Hocini M, Haissaguerre M, Jaïs P. Non-invasive mapping of cardiac arrhythmias. Curr Cardiol Rep. 2015;17(8):60.