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
- Euthyroid
She was euthyroid; ECG revealed regular rhythm with inverted P waves, short PR interval, absent delta waves with normal QRS morphology (Figure 1). Diagnosed as a case of LGL syndrome and put on tab atenolol 50 mg OD. [ncbi.nlm.nih.gov]
Cardiovascular
- 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]
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]
Although tachycardia, along with increased stroke volume, enables cardiac output to meet demands in exercise, a very fast tachycardia is inefficient and may cause compromise. [patient.co.uk]
- 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]
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]
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]
[…] for Disease, Diagnosis I45.6 ICD Code an Classification Pre-excitation syndrome Chapter, Outline, Main Categories, Parent Concepts or Top Concepts: I00-I99 Diseases of the circulatory system (I00-I99)|I30-I52 Other forms of heart disease (I30-I52)|I45 [averbis.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]
- 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]
Psychiatrical
- Suggestibility
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]
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]
Electrophysiologic evidence suggestive of dual A V transmission (atrium to His bundle) is presented in a patient with short P R, narrow QRS, and paroxysmal supraventricular tachycardia. [scholars.northwestern.edu]
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
However, none of the identified short PR interval mechanisms is necessary for the generation of LGL tachycardias. [emedicine.com]
Short PR interval PR interval < 120 msec (< 3 small squares). P wave is normal: upright in leads II, III and avF. QRS width is normal. [metealpaslan.com]
Case 1, with short PR interval and narrow QRS complexes had a short AH interval and intermittent right bundle-branch block. [ncbi.nlm.nih.gov]
P Wave
- Inverted P Wave
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. [symptoma.com]
She was euthyroid; ECG revealed regular rhythm with inverted P waves, short PR interval, absent delta waves with normal QRS morphology (Figure 1). Diagnosed as a case of LGL syndrome and put on tab atenolol 50 mg OD. [ncbi.nlm.nih.gov]
Diagnostic criteria include PR interval of no more than 120 ms, normal QRS complex duration, a normal or inverted P wave, and paroxysmal supraventricular tachycardia (PSVT) but not atrial fibrillation or flutter. [patient.co.uk]
Other ECG Findings
- Ischemic Changes
His ECG does not show typical ischemic changes. Patients with stable angina may have normal ECGs during the angina-free period. [metealpaslan.com]
Treatment
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]
Lown-Ganong-Levine syndrome requires treatment only if the rapid heart rate causes symptoms, or an abnormal heart rhythm occurs. [freemd.com]
Click here for example ECG and further information Links: treatment prophylaxis [gpnotebook.co.uk]
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 [ 7 ]. However, this is unusual, and it is normally far more benign and can be controlled by pharmacological means. [patient.co.uk]
Prognosis Prognosis appears to be good in patients with Lown-Ganong-Levine syndrome. [ncbi.nlm.nih.gov]
Etiology
[…] 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]
Etiology Wolff-Parkinson-White (WPW) is commonly recognized as the classic example of pre-excitation, characterized by the presence of a delta wave with the presence of a short PR interval and a broad QRS complex on ECG. [ncbi.nlm.nih.gov]
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]
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]
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.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]
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
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]
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]
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.