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2.1
Atrial Tachyarrhythmia with Short PR Interval
Lown-Ganong-Levine Syndrome

Presentation

Presentation History The history is of bouts of tachycardia that may present as rapid palpitations. It most often starts in early adulthood but can present in childhood. It tends to get less frequent with passing years. [patient.co.uk]

Abstract A short A-V conduction time, whether present with normal or with abnormal QRS complex, is associated with an increased incidence of paroxysmal rapid heart action. [dx.doi.org]

When the "concertina" effect was present, the direction of the terminal vectors remained unchanged. In four patients with the Lown-Ganong-Levine syndrome, the PSE loop closed, and in three patients, a small opening was present. [ncbi.nlm.nih.gov]

Diagnosis of Lown-Ganong-Levine syndrome is primarily by excluding other arrhythmias by an ECG [1] Rate: 100 to 250 bpm (atrial); ventricular varies Rhythm: regular, narrow QRS complex usually; irregular (ectopic foci) may have wide QRS complex if aberrancy present [explainmedicine.com]

Entire Body System

  • Asymptomatic

    The broad complexes seen in the asymptomatic individual with WPW are not a feature of LGL. [wikidoc.org]

    Patients may remain asymptomatic. Episodes of paroxysmal palpitation (atrial flutter, supraventricular tachycardia) may be associated with shortness of breath, signs of ventricular failure, and syncope. [accessanesthesiology.mhmedical.com]

    The ECG above belongs to an apparently healthy 39 years-old asymptomatic man. Short PR interval is accompanied by right bundle branch block. PR interval is 110 msec. Click here for a more detailed ECG ECG 9b. [metealpaslan.com]

    Most patients are asymptomatic and need no further therapy. SECOND-DEGREE HEART BLOCK Second-degree heart block is characterized by intermittent failure of conduction to the ventricle. It is classified further into Mobitz type I or II block. [neoreviews.aappublications.org]

  • Congestive Heart Failure

    The causes of atrial tachycardia include chronic hypertension, congestive heart failure, valvular heart disease and simply aging of the heart. [healio.com]

    The infant is often noted to be irritable, to not tolerate feedings, or to demonstrate evidence of congestive heart failure. Infants often have a history of not behaving as usual for 1 or 2 days. An intercurrent febrile illness is often observed. [rjmatthewsmd.com]

    heart failure Cardiac enzymes Myocardial infarction or ischemia Diagnostics Chest radiography Cardiomegaly Congestive heart failure or cardiomyopathy Holter monitor or event recorder Capture aberrant rhythm, frequency, duration Type of tachyarrhythmia [aafp.org]

    In addition to age, other common cardiac precursors include a history of congestive heart failure, valvular heart disease, hypertension, and coronary artery disease. [guwsmedical.info]

    Other persons become weak and dizzy, and then can develop symptoms of congestive heart failure. The heart cannot work effectively if the ventricular rate is too rapid. [nurseslearning.com]

  • Anemia

    They are sinus tachycardias related to enhanced sinus automaticity, abnormal autonomic function (dysautonomia), or physiologic reflexes Differentials Atrial Fibrillation Atrial Flutter Atrioventricular Nodal Reentry Tachycardia Chronic Anemia Paroxysmal [emedicine.medscape.com]

    anger, stress MI Fever Anxiety PE Decreased effective circulating volume Sepsis: to augment CO to compensate for low SVR Systolic heart failure: to compensate for low stroke volume Endocrine abnormalities: hyperthyroidism, pheochromocytoma Hypoxia Anemia [errolozdalga.com]

    The following appears in the “Foundational Facts: Understanding Sinus Tachycardia” box on page 125: “Sinus tachycardia is caused by external influences on the heart, such as fever, anemia, hypotension, blood loss, or exercise. [ems12lead.com]

    We must also say that the LQT can be produced by external agents (non-genetic) as drugs and some systemic disorders, such as anemia. [medcraveonline.com]

    Sinus tachycardia rarely exceeds 200 beats/min; it should not be treated for itself, but its causes must be explored (e.g., exercise, fever, anemia, hyperthyroidism), and some of these (e.g., fever, anemia, hyper-thyroidism) may require therapy. 2.2.2 [guwsmedical.info]

  • Atrial Septal Defect

    Mutation in myosin heavy chain 6 causes atrial septal defect. Nat. Genet. 37, 423–428 (2005). 41. Emilsson, V. et al. Genetics of gene expression and its effect on disease. Nature 452, 423–428 (2008). 42. Maier, S.K. et al. [nature.com]

    There is no specific treatment – and it may often be caused by an atrial septal defect Left Bundle Branch Block (LBBB) Left Bundle Branch Block (LBBB) – basic waveform Left Bundle Branch Block (LBBB) on a full ECG Usually indicates left sided heart disease [almostadoctor.co.uk]

Liver, Gall & Pancreas

  • Hepatomegaly

    If the episode has been untreated for several hours, the patient often has poor perfusion, hepatomegaly, and cardiac failure. The child is usually anxious but hemodynamically stable. Tachypnea often accompanies the tachycardia. [rjmatthewsmd.com]

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]

    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]

    Antidromic AVRT: short RP interval (< 100 msec); regular, wide QRS complex (≥ 120 msec); delta waves seen with normal sinus rhythm and tachycardia; concealed accessory pathways do not show delta waves [2] Other Supraventricular tachycardias- atrial tachycardia [explainmedicine.com]

  • Heart Disease

    Stay on top of the latest advances in molecular imaging, intravascular ultrasound, cardiovascular regeneration and tissue engineering, device therapy for advanced heart failure, atrial fibrillation management, structural heart disease, Chagasic heart [books.google.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. [dx.doi.org]

    […] 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]

    History of cardiac disease (heart failure, valve disease, congenital heart disease, coronary heart disease). Features of the history or results of further investigation may prompt an urgent referral. [patient.info]

  • Hypertension

    Hypertensive heart disease - Hypertensive nephropathy - Secondary hypertension ( Renovascular hypertension ) Ischaemic heart disease Angina pectoris ( Prinzmetal's angina ) - Myocardial infarction (heart attack) - Dressler's syndrome Pulmonary circulation [wikidoc.org]

    The ECG above belongs to a 73 years-old man with chronic hypertension. He complained of typical effort angina. His ECG shows increased voltage suggestive of left ventricular hypertrophy, strain pattern and short PR interval. [metealpaslan.com]

    The causes of atrial tachycardia include chronic hypertension, congestive heart failure, valvular heart disease and simply aging of the heart. [healio.com]

    PE) Sympathetic tone (e.g. postop, pain) Infection Valvular disease Post-cardiac surgery Valvular disease Atrial enlargement (e.g. secondary to hypertension) Hyperthyroidism Management Rate control: can be difficult, given that ventricular rate drops [errolozdalga.com]

    Genome-wide association study of blood pressure and hypertension. Nat. Genet. 41, 677–687 (2009). 26. Rabert, D.K. et al. A tetrodotoxin-resistant voltage-gated sodium channel from human dorsal root ganglia, hPN3/SCN10A. Pain 78, 107–114 (1998). 27. [nature.com]

Workup

Workup Laboratory Studies Workup is directed at determining the cause of tachycardia. Lown-Ganong-Levine syndrome (LGL) is an outdated diagnosis, and as such no workup is directed at making this diagnosis. [emedicine.medscape.com]

In the acute setting, a standard workup for tachycardia is indicated i.e. ECG, lytes, TSH. In patients who have dyspnea, a chest X-ray should be obtained. [emdocs.net]

WORKUP Lab Studies: The extent of the workup is determined by the acuity of the patient’s illness. [rjmatthewsmd.com]

Table 4 lists items to include in a focused examination and diagnostic workup. Enlarge Print Table 4. [aafp.org]

X-Ray

  • Pericardial Effusion

    effusion - Cardiac tamponade Endocardium / heart valves Endocarditis - mitral valve ( regurgitation, prolapse, stenosis ) - aortic valve ( stenosis, insufficiency ) - pulmonary valve ( stenosis, insufficiency ) - tricuspid valve ( stenosis, insufficiency [wikidoc.org]

    effusion ( Hemopericardium, Cardiac tamponade ) Myocardium Myocarditis ( Chagas disease ) Cardiomyopathy : Dilated ( Alcoholic ) · Hypertrophic · Restrictive ( Loeffler endocarditis, Cardiac amyloidosis, Endocardial fibroelastosis ) Arrhythmogenic right [dictionnaire.sensagent.leparisien.fr]

PR Interval

  • Short PR Interval

    However, none of the identified short PR interval mechanisms is necessary for the generation of LGL tachycardias. [emedicine.com]

    […] communication from the atria to the ventricles atrial tachyarrhythmia with short PR interval syndrome of short P-R interval, normal QRS complexes, and supraventricular tachycardias LGL syndrome Statements Identifiers Sitelinks Wikipedia (10 entries) [wikidata.org]

    Five additional family members had short PR intervals with normal QRS, and eight other family members had borderline short PR intervals. The mode of inheritance appeared to be autosomal dominant with varying expressivity. [scholars.northwestern.edu]

    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]

Rhythm

  • Torsades De Pointes

    Torsade de Pointes When polymorphic ventricular tachycardia occurs in the presence of prolonged QT intervals (congenital or required), it is termed torsade de pointes. [guwsmedical.info]

    de pointes · Catecholaminergic polymorphic · Accelerated idioventricular rhythm Premature contraction Atrial · Ventricular Pre-excitation syndrome Wolff-Parkinson-White · Lown-Ganong-Levine Flutter/ fibrillation Atrial flutter · Ventricular flutter · [dictionnaire.sensagent.leparisien.fr]

    Ventricular arrhythmias associated with long QT syndromePolymorphic VT consistent with torsades de pointes. • Congenital • Acquired — Heart block — Medication — Other Ventricular arrhythmias associated with Brugada syndromePolymorphic VT in the absence [onlinejacc.org]

    V-tach that changes orientation (changes QRS axis) regularly over a period of many beats is called Torsades de pointes. [madsci.com]

    de Pointes Ventricular Tachycardia Wolff-Parkinson-White Syndrome Author Adam S Budzikowski, MD, PhD, FHRS Assistant Professor of Medicine, Division of Cardiovascular Medicine, Electrophysiology Section, State University of New York Downstate Medical [emedicine.medscape.com]

  • Premature Atrial Contractions

    This is referred to as an “ectopic atrial rhythm” or “ectopic P wave.” Ectopic P waves are also commonly seen in multifocal atrial tachycardia, or MAT, wandering atrial pacemaker, and premature atrial contractions, or PACs. [healio.com]

    Ectopic supraventricular rhythms include Atrial premature beats Atrial tachycardia Multifocal atrial tachycardia Nonparoxysmal junctional tachycardia Wandering atrial pacemaker Atrial premature beats (APB), or premature atrial contractions (PAC), are [merckmanuals.com]

    Premature atrial contractions 3. Nonconducted premature atrial contractions 4. Atrial tachycardia 5. Paroxysmal atrial tachycardia 6. Supraventricular tachycardia 7. Atrial flutter 8. Atrial fibrillation IX. Wandering Atrial Pacemaker A. [andrews.edu]

    Premature Atrial Contractions or Atrial Premature Beats (PAC's or APB's) : This is a single premature beat meaning that it occurs earlier than expected. [jan.ucc.nau.edu]

    However, if a premature atrial contraction (PAC) arrives while the fast pathway is still refractory, the electrical impulse will be directed solely down the slow pathway (1). [lifeinthefastlane.com]

  • Atrioventricular Dissociation

    Atrioventricular dissociation is present VT present; stop and treat Proceed to 4 4. [aafp.org]

    Specific ECG findings for junctional tachycardias (i) inverted P waves in inferior leads (II, III, aVF) (ii) narrow QRS (iii) atrioventricular dissociation 7. [clinicaladvisor.com]

Blocks

  • Right Bundle Branch Block

    Case 1, with short PR interval and narrow QRS complexes had a short AH interval and intermittent right bundle-branch block. [heart.bmj.com]

    block, unspecified I45 Other conduction disorders I45.0 Right fascicular block I45.1 Other and unspecified right bundle-branch block I45.10 Unspecified right bundle-branch block I45.19 Other right bundle-branch block I45.2 Bifascicular block I45.3 Trifascicular [icd10data.com]

    bundle-branch block. ( 287918 ) 1979 18 Lown-Ganong-Levine syndrome. ( 679452 ) Manning G.W. 1978 19 Abnormalities of atrioventricular conduction in patients with the Lown-Ganong-Levine syndrome. ( 747919 ) Pavlovic J....BelA! [malacards.org]

    International Classification of Diseases, Tenth Revision, Clinical Modification.I45.6 Pre-excitation syndrome and Lown Ganong Levine syndrome I45.10 ECG: complete right bundle branch block I45.9 Aberrantly conducted complex (disorder) [averbis.com]

    Short PR interval is accompanied by right bundle branch block. PR interval is 110 msec. Click here for a more detailed ECG ECG 9b. The same patient's ECG summary is seen above. ECG 10. [metealpaslan.com]

  • AV Block Mobitz I

    block (PR interval 340ms) Second degree AV block (Mobitz I) with prolonged PR interval Second degree heart block, Mobitz type I (Wenckebach phenomenon). [lifeinthefastlane.com]

Treatment

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]

This era was followed by improved environmental factors which operate to produce methods of medical treatment, introduction congenital cardiac malformations. [books.google.com]

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]

Treatment of PAT As we mentioned earlier, PAT will often stop spontaneously and requires no treatment. IN more severe cases, which are either prolonged or case clinical symptoms, treatment is required. [nurseslearning.com]

Treatment 1. No treatment if asymptomatic 2. Treatment is directed toward the underlying cause 3. Cause may be unrecognized XI. Nonconducted PAC A. [andrews.edu]

Prognosis

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]

Advise about driving where relevant. [ 7 ] Prognosis [ 2 ] Prognosis is dependent upon the cause. It is important to diagnose the serious causes of palpitations. [patient.info]

Prognosis Prognosis appears to be good in patients with Lown-Ganong-Levine syndrome. [ncbi.nlm.nih.gov]

Long-term prognosis of pre-excitation and WPW syndrome The majority of all patients with pre-excitation will experience AVRT. [ecgwaves.com]

Etiology

Their etiology is not precisely century with the first clinical recognition of known, so we have included chapters that dis symptoms and patterns for diagnosis of car cuss many aspects of congenital cardiac mal diovascular diseases. [books.google.com]

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]

In Western countries, myocarditis or pericarditis (most commonly of viral etiology) are occasionally associated with arrhythmias. [guwsmedical.info]

This type of tachycardia may be caused by hypomagnesemia (among other etiologies), and is important to recognize because its treatment may differ from standard ventricular tachycardia. [madsci.com]

Etiology: This is a conduction defect in either the right or left branches of the bundle of HIS. Usually, the cause is tissue damage. Clinical Implications: The arrhythmia itself is not dangerous. [nurseslearning.com]

Epidemiology

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]

“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]

Epidemiology The Lown-Ganong-Levine pattern has been reported in a wide age range of patients and has not been shown to display increased incidence in one particular sex or ethnic background. [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. [en.wikipedia.org]

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]

Cijan A. 1983 12 The Lown-Ganong-Levine syndrome. ( 6182544 ) Castellanos A....Myerburg R.J. 1982 13 Analysis of anterograde and retrograde fast pathway properties in patients with dual atrioventricular nodal pathways: observations regarding the pathophysiology [malacards.org]

This number compares to an incidence of less than 0.5% in the general population without shortened PR intervals.[1] Pathophysiology Unlike other pre-excitation syndromes, there is no research currently available that explains well the pathophysiology [ncbi.nlm.nih.gov]

Prevention

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]

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]

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]

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]

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