Lenegre disease refers to disturbances in the cardiac conduction network that typically lead to atrioventricular (AV) and bundle branch block. This disease is a result of genetic and age-related processes. Patients are at risk for life-threatening complications such as heart failure and sudden cardiac death.
Presentation
Lenegre disease, also known as progressive cardiac conduction defect (PCCD), is described as an alteration in the cardiac conduction network [1] [2]. It is believed that this disease is caused by a mutation in the SCN5A gene in combination with age-related degeneration and fibrosis of the His-Purkinje system and its branches [3] [4]. An electrocardiogram (EKG) performed in affected individuals will reveal a conduction delay in the His-Purkinje tissues and varying degrees of arterioventricular (AV) block and differing types of branch block [5] [6]. The cardiac structure and function are typically normal in these patients.
Lenegre disease is considered as one of the most predominant conductive disturbances and is a common cause of pacemaker implantation worldwide. Moreover, it is diagnosed in patients below the age of 50 who have unexplained conduction disease but normal heart structure and likely a family history positive for Lenegre disease [7].
While some patients exhibit no symptoms, others experience dyspnea, lightheadedness, dizziness, abdominal pain, and syncope. As a result of complete AV heart block, complications such as left ventricular dilatation, heart failure, and sudden cardiac death may ensue. The latter commonly occurs in advanced cases of first-degree AV block accompanied by a bi- fascicular block as well as patients with symptomatic AV block. However, pacemaker implantation improves the prognosis significantly.
Entire Body System
- Asymptomatic
Diagnostic Criteria Many patients with type 1 ECG pattern are asymptomatic. [aerjournal.com]
The tragic nature of sudden death in young, previously asymptomatic athletes, has led to considerable efforts aimed at prevention. [gmj.ir]
[…] conductive system.The right bundle branch may be initially and mostly involved,followed by the left anterior fascicle and the highly or complete atrioventricular block found in severe cases.Patients with single or double bundle branch block frequently appear asymptomatic [en.cnki.com.cn]
His mother (75-year-old) was a carrier of the mutation but was asymptomatic and had a normal ECG both at baseline and following flecainide challenge. [revespcardiol.org]
- Fatigue
The case of a 22-year-old white male without known heart disease who presented with activity related lightheadedness at age 19 and dizziness and fatigue at age 21 is described. [scholars.northwestern.edu]
Abstract The case of a 22-year-old white male without known heart disease who presented with activity related lightheadedness at age 19 and dizziness and fatigue at age 21 is described. [ncbi.nlm.nih.gov]
Risks with CBD include: suicidal thoughts, suicide attempts, feelings of agitation, new or worsening depression, aggression and panic attacks in addition to liver injury, which can cause nausea, vomiting, abdominal pain, fatigue, anorexia, jaundice and [mdmag.com]
Mobitz Type I patients may experience dizziness, fatigue, and intolerance to exercise due to the delay of the electrical impulses in the heart. [vacardio.com]
Presentation Bradycardia may be asymptomatic but can present with syncope, fatigue or dizziness. [patient.info]
- Unconsciousness
During a seizure a child will become unconscious. They may look blank or stare and their eyelids may flutter. They may not respond to what is happening around them or be aware of what they are doing. Seizures respond well to medication. [epilepsysociety.org.uk]
This type of seizure is associated with a period of unconsciousness usually marked by unresponsive staring. Absence seizures usually begin and end abruptly and the affected individual usually resumes activity with no memory of the episode. [rarediseases.org]
Gastrointestinal
- Nausea
Risks with CBD include: suicidal thoughts, suicide attempts, feelings of agitation, new or worsening depression, aggression and panic attacks in addition to liver injury, which can cause nausea, vomiting, abdominal pain, fatigue, anorexia, jaundice and [mdmag.com]
Second-degree heart block might cause: Dizziness Fainting The feeling that your heart skips beats Chest pain Difficulty breathing or shortness of breath Nausea Fatigue Third-degree heart block, which can be fatal, might cause Intense tiredness Irregular [columbiadoctors.org]
Establish whether there is a history of faintness, dizziness, nausea or chronic fatigue. It is important to ask about medication, as drugs are an important cause of bradycardia. [patient.info]
Within an hour before cardiac arrest, some people have chest pain, shortness of breath, nausea, or vomiting. Call 9-1-1 right away if someone has signs or symptoms of sudden cardiac arrest. [nhlbi.nih.gov]
[…] foot contraction deformities; Flexion contractures; Pes cavus; Trophic limb changes Resp: Laryngeal abnormalities; Neonatal inspiratory stridor Facies: Peculiar facies Neck: Short neck Thorax: Narrow shoulders; Protruding chest GI: Chronic diarrhea; Nausea [findzebra.com]
- Vomiting
Risks with CBD include: suicidal thoughts, suicide attempts, feelings of agitation, new or worsening depression, aggression and panic attacks in addition to liver injury, which can cause nausea, vomiting, abdominal pain, fatigue, anorexia, jaundice and [mdmag.com]
[…] contraction deformities; Flexion contractures; Pes cavus; Trophic limb changes Resp: Laryngeal abnormalities; Neonatal inspiratory stridor Facies: Peculiar facies Neck: Short neck Thorax: Narrow shoulders; Protruding chest GI: Chronic diarrhea; Nausea; Vomiting [findzebra.com]
Within an hour before cardiac arrest, some people have chest pain, shortness of breath, nausea, or vomiting. Call 9-1-1 right away if someone has signs or symptoms of sudden cardiac arrest. [nhlbi.nih.gov]
Phenytoin Inflammatory disease Electrolyte disturbances Myocarditis Hypothyroidism Infiltrative disorders Sleep apnea Collagen vascular diseases Hypoxia Musculoskeletal disorders Hypothermia Cardiothoracic surgery Increased vagal tone Valve replacement Vomiting [revespcardiol.org]
Cardiovascular
- Heart Block
block (disorder)|Complete heart block|Complete heart block NOS|Dressler beats|ECG: complete atrioventricular block|EKG: complete atrioventricular block|Electrocardiographic complete atrioventricular block|Electrocardiographic complete atrioventricular [averbis.com]
Lev's disease (or Lenegre-Lev syndrome) is an acquired complete heart block due to idiopathic fibrosis and calcification of the electrical conduction system of the heart. [diki.pl]
[…] type IA, heart block, nonprogressive, progressive familial heart block, type IA, heart block, progressive familial, type 1, Lenegre's syndrome, bundle branch block, progressive familial heart block caused by mutation in SCN5A, PFHB1A References: OMIM [monarchinitiative.org]
Left bundle branch block masquerading as right bundle branch block. Am Heart J. 1954; 47(3): 383-393. Lenègre J. Etiology and pathology of bilateral bundle branch block in relation to complete heart block. Prog Cardiovasc Dis 1964; 6: 409-444 Lev M. [litfl.com]
Lev: The pathology of complete atrioventricular block. The American Journal of Medicine, New York, 1964, 37: 742-748 J. Lenègre: Etiology and pathology of bilateral bundle branch block in relation to complete heart block. [whonamedit.com]
- Heart Disease
American Heart Journal, 94(4), 479-485. https://doi.org/10.1016/S0002-8703(77)80043-4 @article{0f5aeea346224edcaceb96c8111534e0, title = "Lenegre's disease in youth", abstract = "The case of a 22-year-old white male without known heart disease who presented [scholars.northwestern.edu]
Most congenital heart disease will be evident in early life, presenting with cyanosis, heart murmur, congestive heart failure or shock. [books.google.com]
disease Rheumatic heart disease Myocarditis or cardiomyopathy Degenerative disease of the conduction system Congenital heart disease e.g. atrial septal defect ECG Diagnostic criteria Broad QRS >120ms RSR pattern in V1-3 (‘M’ shaped QRS) Caused by a delay [dundeemedstudentnotes.wordpress.com]
[…] syndrome|Lev's disease|Lev's syndrome|Lev's syndrome (disorder)|Lev-Lenègre disease|Progressive cardiac conduction defect|Progressive familial heart block, type IA|Third degree atrioventricular block|Third degree block|Third degree heart block|Ventricular [averbis.com]
- Hypertension
aortic stenosis, aortic valve replacement, Chagas disease, coronary artery disease, dilated cardiomyopathy, endocarditis, exercise-induced LBBB, heart failure, hemochromatosis, hyperkalemia, hypertension, hypertensive cardiomyopathy, hypertrophic cardiomyopathy [wikidoc.org]
Structural heart disease Chronically high right ventricular pressure (i.e. cor pulmonale) Acutely high right ventricular pressure with stretch (i.e. pulmonary embolism) Myocardial ischemia Myocardial infarction Myocardial inflammation (i.e. myocarditis) Hypertension [dimasyusuf.com]
[…] this eliminates the normal septal Q waves in the lateral leads INCOMPLETE Left Bundle Branch Block - Normal QRS duration, but otherwise looks like a left bundle branch block Causes of Left Bundle Branch Block - Ischaemia - Aortic stenosis or severe hypertension [derangedphysiology.com]
[…] finally a terminal extreme bradycardia Disease associations Although sometimes termed “idiopathic” fibrosis of the conducting system, degenerative changes seen are often a result of ischaemic heart disease, prior myocardial infarction, or long-standing hypertension [litfl.com]
Aortic stenosis Ischaemic heart disease Hypertension Dilated cardiomyopathy Anterior MI Primary degenerative disease (fibrosis) of the conducting system (Lenegre disease) Hyperkalaemia Digoxin toxicity Older people are more likely to have LBBB due to [dundeemedstudentnotes.wordpress.com]
Neurologic
- Dizziness
The case of a 22-year-old white male without known heart disease who presented with activity related lightheadedness at age 19 and dizziness and fatigue at age 21 is described. [scholars.northwestern.edu]
Abstract The case of a 22-year-old white male without known heart disease who presented with activity related lightheadedness at age 19 and dizziness and fatigue at age 21 is described. [ncbi.nlm.nih.gov]
You may feel dizzy. Mobitz type II: The electrical signals sometimes get to the ventricles, and sometimes they do not. You may require a pacemaker, a small machine that provides regular electrical pulses to manage your heart’s rhythm. [columbiadoctors.org]
While some patients exhibit no symptoms, others experience dyspnea, lightheadedness, dizziness, abdominal pain, and syncope. [symptoma.com]
In the age group of interest for this book of 11-18 years, the most common presenting issues were rhythm related (23%) and chest pain (23%), followed by syncope or dizziness (19%) and heart murmur (12%). [books.google.com]
- Vertigo
[…] disorders Irregular heart beat Irregular heartbeat [ more ] 0011675 Bundle branch block 0011710 Congestive heart failure Cardiac failure Cardiac failures Heart failure [ more ] 0001635 Dyspnea Trouble breathing 0002094 Syncope Fainting spell 0001279 Vertigo [rarediseases.info.nih.gov]
- Aura
Missing: aura prykushuvannya tongue (epilepsy). Faces in epilepsy blue- purple, after the attack - the pale. At the MAC - first pale, while the court - cyanotic, and after the attack - red 24. [slideshare.net]
Workup
Patients with the symptoms described above (such as syncope) or incidental EKG findings suggestive of a cardiac conductive defect warrant a thorough evaluation. In addition to ascertaining the personal history, the workup should include a detailed family history of diseases and manifestations such as syncope or sudden death. Patients should also undergo a complete physical exam with a focus on the cardiovascular system. Finally, affected individuals should be assessed with specific studies like electrocardiogram, echocardiogram etc.
Electrocardiogram (EKG)
Findings associated with Lenegre disease include prolonged PR interval, wide QRS complexes, right and/or left bundle branch block, as well as disturbances in other conductive tissues. An exercise stress test in patients with a complete AV block may reveal arrhythmias such as ventricular tachycardia and Torsade de Pointes.
Imaging
An echocardiogram and/or cardiac magnetic resonance imaging (MRI) are performed to assess the contractile function and structure of the heart and to rule out underlying abnormalities such as cardiomyopathy and congenital heart disease [7].
Other
Genetic testing is advised in young individuals with Lenegre disease.
Axis
- Left Axis Deviation
axis deviation (= left anterior fascicular block) First degree AV block Example 2 Incomplete Trifascicular Block: Right bundle branch block Left axis deviation (= left anterior fascicular block) First degree AV block Example 3 Complete Trifascicular [lifeinthefastlane.com]
Case 5 Answer Rate -about 40 Rhythm - second degree block type 2 Intervals - prolonged qrs in V2 consistent with right bundle branch block Axis - left axis deviation from anterior fascicular block Hypertrophy - none Infarct - none Clinical correlation [inside.fammed.wisc.edu]
Left axis deviation: an electrocardiographic mortality study. [ahajournals.org]
left axis deviation (Belhassen type) or in bundle branch reentrant ventricular tachycardia. [dimasyusuf.com]
Blocks
- Right Bundle Branch Block
branch block masquerading as right bundle branch block” and described 4 cases which probably corresponded (especially case no. 1) to bilateral bundle branch block – a pattern of complete left bundle branch block in the standard leads and of complete [litfl.com]
3 Complete Trifascicular Block: Right bundle branch block Left axis deviation (Left anterior fascicular block) Third degree heart block Related Topics Left bundle branch block LBBB Right Bundle Branch Block RBBB Left anterior fascicular blo c k LAFB [lifeinthefastlane.com]
[…] hemiblock 426.3 Other left bundle branch block 426.4 Right bundle branch block 426.50 Bundle branch block, unspecified 426.51 Right bundle branch block and left posterior fascicular block 426.52 Right bundle branch block and left anterior fascicular block [healthprovidersdata.com]
Pseudo right bundle branch block Brugada syndrome Arrhythmogenic right ventricular cardiomyopathy [dimasyusuf.com]
The epidemiology of right bundle branch block and its association with cardiovascular morbidity--the Reykjavik Study. Eur Heart J 1993; 14:1590. Fahy GJ, Pinski SL, Miller DP, et al. Natural history of isolated bundle branch block. [medics4medics.nl]
- Left Anterior Fascicular Block
1 Incomplete Trifascicular Block: Right bundle branch block Left axis deviation (= left anterior fascicular block) First degree AV block Example 2 Incomplete Trifascicular Block: Right bundle branch block Left axis deviation (= left anterior fascicular [lifeinthefastlane.com]
[…] hemiblock 426.3 Other left bundle branch block 426.4 Right bundle branch block 426.50 Bundle branch block, unspecified 426.51 Right bundle branch block and left posterior fascicular block 426.52 Right bundle branch block and left anterior fascicular block [healthprovidersdata.com]
[…] bundle branch block, left anterior fascicular block / hemiblock or left posterior hemiblock, prolonged PR interval or complete AV block with broad QRS complexes). [orpha.net]
[…] atrioventricular block I44.4 Left anterior fascicular block I44.5 Left posterior fascicular block I44.6 Other and unspecified fascicular block I44.60 Unspecified fascicular block I44.69 Other fascicular block I44.7 Left bundle-branch block, unspecified [icd10data.com]
Left anterior fascicular block MedGen UID: 75547 •Concept ID: C0264912 • Disease or Syndrome Conduction block in the anterior division of the left bundle branch of the bundle of His. [ncbi.nlm.nih.gov]
- Bifascicular Block
Incomplete trifascicular block Bifascicular block + 1st degree AV block (most common) Bifascicular block + 2nd degree AV block RBBB + alternating LAFB / LPFB Complete trifascicular block Bifascicular block + 3rd degree AV block NB. [lifeinthefastlane.com]
This older patient has a bifascicular block combined with a Mobitz II block and this setting often antedates complete heart block. [inside.fammed.wisc.edu]
A high incidence of sudden death is observed in patients with either first-degree AV block in association with bifascicular block or in those with symptomatic advanced AV block. [orpha.net]
This may be preceded by first-degree AV block, second-degree AV block, bundle branch block, or bifascicular block. In addition, acute myocardial infarction may present with third-degree AV block. [findzebra.com]
- Third Degree Atrioventricular Block
[…] syndrome|Lev's disease|Lev's syndrome|Lev's syndrome (disorder)|Lev-Lenègre disease|Progressive cardiac conduction defect|Progressive familial heart block, type IA|Third degree atrioventricular block|Third degree block|Third degree heart block|Ventricular [averbis.com]
Third-degree AV block In conclusion, we reported a case of third-degree atrioventricular block in a young and otherwise healthy patient following cannabis use. [hindawi.com]
[…] ablation Complete atrioventricular block Complete atrioventricular block (heart beat disorder) Complete atrioventricular block due to atrioventricular nodal ablation Complete AV block due to AV nodal ablation Applies To Third degree atrioventricular [icd9data.com]
(Third Degree Heart Block, Complete Heart Block) (see Third Degree Atrioventricular Block, [[Third Degree Atrioventricular Block]]) Epidemiology Fibrosis/Sclerosis of the Conduction System : accounts for 50% of cases of all atrioventricular blocks Ischemic [mdnxs.com]
degree atrioventricular block. a Increasing less than 100 beats/min during limited exercise test. b Not shortening with hyperventilation or limited exercise test. c A recent study suggests athletes with a QTc > 500ms should be recommended not to participate [en.ecgpedia.org]
- First-Degree Atrioventricular Block
First-degree atrioventricular block caused by intranodalconduction delay 15. First-degree atrioventricular block secondary to His-Purkinjesystem (HPS) disease 16. [slideshare.net]
Similar Codes ICD-9 Code ICD-9 Description 426.10 Atrioventricular block, unspecified 426.11 First degree atrioventricular block 426.12 Mobitz (type) II atrioventricular block 426.13 Other second degree atrioventricular block 426.2 Left bundle branch [healthprovidersdata.com]
[…] system See Cardiac Physiology, [[Cardiac Physiology]] Classification of Atrioventricular Blocks First Degree Atrioventricular Block (First Degree Heart Block) (see First Degree Atrioventricular Block, [[First Degree Atrioventricular Block]]) Second Degree [mdnxs.com]
It is important to note that in first degree AV block, no actual block occurs. First-degree atrioventricular block. The P waves are buried within the T waves. The PR interval is 280 milliseconds (about 7 small squares). [acls.com]
Neuromuscular diseases (eg, myotonic muscular dystrophy, Kearns-Sayre syndrome, etc.) with first-degree atrioventricular block IIb B 1. Asymptomatic first-degree atrioventricular block III C 2. [revespcardiol.org]
Other ECG Findings
- Abnormal ECG
It's the perfect book to turn to for clear and clinically relevant guidance on all of today’s ECG applications. Comprehensively and expertly describes how to capture and interpret all normal and abnormal ECG findings in adults and children. [books.google.com]
Abnormal ECG intervals including P wave duration, PR or QRS duration may be commonly observed. [aerjournal.com]
The ECG of 203 football players showed no abnormality. The rest ECG of 55 football players had some degree of abnormalities that were listed in Table 2. [gmj.ir]
Treatment
Skip to content Home About Us Team Log In News Videos Treatments Clinical Trials Access Programs Diseases Events Networks All News People Treatment Research Finance All Access Programs Main Menu Subscribe for Free Newsletter Email * Type * * Required [checkorphan.org]
Modalities 729 Treatment Modalities 731 Clinical Role of Antiarrhythmic Drugs in the Prevention of Sudden Death 733 Nonantiarrhythmic Drugs in Sudden Death Prevention 760 The Implantable Cardioverter Defibrillator Technical and Clinical Considerations [books.google.com]
J Child Neurol, 11(1): 31-4 [ CR ] [ PM ] [ EČ ][ GS ] Conry, J.A. (2004) Pharmacologic treatment of the catastrophic epilepsies. [scindeks.ceon.rs]
Biological pacemakers as future treatment for complete heart block. Electronic pacemakers are the main treatment for some patients who have conduction disorders such as complete heart block. [nhlbi.nih.gov]
Prognosis
Most cardiac disorders present with different symptoms and signs, require a different diagnostic and therapeutic approach, and also have a different prognosis in the elderly as compared to younger patients. [books.google.com]
However, pacemaker implantation improves the prognosis significantly. Patients with the symptoms described above (such as syncope) or incidental EKG findings suggestive of a cardiac conductive defect warrant a thorough evaluation. [symptoma.com]
Prognosis Long-term prognosis overall is unfavourable but variable. [patient.info]
Prognosis There is no genotype-based risk stratification for patients with PCCD. [orpha.net]
Prognosis of Atrioventricular Conduction Block The prognosis of patients with AV conduction disturbances depends on the site of the block, but also particularly on the concomitant or underlying heart disease. [revespcardiol.org]
Etiology
Causes: Structural brain abnormalities (most common cause, 70% of cases) Genetic etiologies (de novo mutations) are expected to account for many of the currently unexplained cases [epilepsydiagnosis.org]
Predominant sex: male = female Pediatric Considerations Rare during pregnancy Etiology and Pathophysiology Medications Digoxin (common) Calcium channel blockers β-Blockers (e.g., sotalol) Clonidine Propafenone (Rythmol), a class IC antiarrhythmic Isoproterenol [unboundmedicine.com]
Etiologies include conduction system disease, drug effects, and myocardial ischemia. In this case the patient was admitted to the hospital with his symptomatci high degree block. [inside.fammed.wisc.edu]
Epidemiology
In the fifth edition of Tresch and Aronow's Cardiovascular Disease in the Elderly, each chapter has been thoroughly updated to provide a comprehensive, yet accessible overview of the epidemiology, pathophysiology, evaluation, and treatment of cardiovascular [books.google.com]
We conclude that the time has come to relieve epidemiology of the French paradox. [epi.umn.edu]
occurs in younger patients (may be hereditary) Physiology : progressive, fibrotic, sclerodegenerative disease of the conduction system Clinical : frequently associated with slow progression to complete heart block Lev’s Disease Epidemiology : occurs [mdnxs.com]
Summary Epidemiology Incidence is estimated at 1:1,000,000 inhabitants per year, and the estimated prevalence is 15/100,000. Lennox-Gastaut syndrome (LGS) represents 5-10% of epileptic patients, and 1-2% of all childhood epilepsies. [orpha.net]
System(s) affected: cardiovascular, nervous Synonym(s): drop attacks; Morgagni, Morgagni-Adams-Stokes; Spens syndrome Epidemiology Incidence Incidence rate of 5–10% for those age >70 years Prevalence Unknown (Overall prevalence of 3rd-degree atrioventricular [unboundmedicine.com]
Pathophysiology
In the fifth edition of Tresch and Aronow's Cardiovascular Disease in the Elderly, each chapter has been thoroughly updated to provide a comprehensive, yet accessible overview of the epidemiology, pathophysiology, evaluation, and treatment of cardiovascular [books.google.com]
Predominant sex: male = female Pediatric Considerations Rare during pregnancy Etiology and Pathophysiology Medications Digoxin (common) Calcium channel blockers β-Blockers (e.g., sotalol) Clonidine Propafenone (Rythmol), a class IC antiarrhythmic Isoproterenol [unboundmedicine.com]
Left Bundle Branch Block Pathophysiology Normally, the septum is activated from left to right, producing small Q waves in the lateral leads (I, aVL and V5-6) In complete LBBB, this is reversed as electrical activity first has to pass through the right [dundeemedstudentnotes.wordpress.com]
[…] disorder caused by SCN5A gene mutation leading to an autosomal dominant hereditary disease that is characterized of a higher incidence rate in males than in females,a lower age of onset(mostly 40 years) and a significant familial aggregation.This has been pathophysiologically [en.cnki.com.cn]
College of Cardiology 41(4): 643-652, February 19 2003 OBJECTIVES The goal of this study was to investigate the genotype-to-phenotype relationship between SCN5A gene mutation and progressive cardiac conduction defect in order to gain insights into the pathophysiologic [eurekamag.com]
Prevention
[…] of Sudden Death 733 Nonantiarrhythmic Drugs in Sudden Death Prevention 760 The Implantable Cardioverter Defibrillator Technical and Clinical Considerations 772 Beyond Sudden Death Prevention Minimizing Implantable Cardioverter Defibrillator Shocks and [books.google.com]
Medicines May Prevent Conduction System Disease in Some Individuals. [nhlbi.nih.gov]
Prevention of cardiovascular disease through diet/exercise Commonly Associated Conditions Myocardial ischemia/acute MI High-degree AV conduction abnormality Atrial standstill Right bundle branch block (RBBB) CCAVB Systemic manifestations of connective [unboundmedicine.com]
Prevention You may sometimes prevent heart block — in your baby, for example. If you are a woman with an autoimmune disease and give birth, your baby has an increased risk of developing heart block. [columbiadoctors.org]
However, your pacemaker may not prevent escape tachycardias, which is a type of fast heart rhythm, so you may also be prescribed medicines to stop this from happening. [bhf.org.uk]
References
- Lev M, Kinare SG, Pick A. The pathogenesis of atrioventricular block in coronary disease. Circulation. 1970;42(3):409-25.
- Lev M, Cuadros H, Milton HP. Interruption of the atrioventricular bundle with congenital atrioventricular block. Circulation. 1971;43(5):703-10.
- Probst V, Kyndt F, Potet F, Trochu JN, Mialet G, Demolombe S, Schott JJ, Baró I, Escande D, Le Marec H. Haploinsufficiency in combination with aging causes SCN5A-linked hereditary Lenegre disease. J Am Coll Cardiol. 2003; 41(4):643–652.
- Schott JJ, Alshinawi C, Kyndt F, et al. Cardiac conduction defects associate with mutations in SCN5A. Nat Genet. 1999; 23(1):20–21.
- Baruteau AE, Probst V, Abriel H. Inherited progressive cardiac conduction disorders. Curr Opin Cardiol. 2015;30(1):33-9.
- Lev M. The pathology of complete atrioventricular block. Prog Cardiovasc Dis. 1964;6:317-26.
- Bharati S, Lev M, Dhingra RC, et al. Electrophysiological and pathological correlations in two cases of chronic second degree atrioventricular block with left bundle branch block. Circulation. 1975;52(2):221-9.