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Lenegre Disease

Lenegre-Lev Syndrome

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.

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]
  • 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]
  • The diagnosis is usually made when detecting a slow pulse or by presentation with fatigue or syncope, though syncope can herald a more ominous course. Fig. 16.3 Complete heart block with junctional escape.[thoracickey.com]
Asymptomatic
  • […] 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]
  • Presentation Bradycardia may be asymptomatic but can present with syncope, fatigue or dizziness.[patient.info]
  • Approximately 2 % of babies born to SSA/SSB   mothers, the majority of whom are often asymptomatic, have CHB; however, the recurrence rate for future offspring may be as high as 17 %.[thoracickey.com]
  • Asymptomatic bifascicular block with first degree AV block is not an indication for pacing (class III).[lifeinthefastlane.com]
  • In some disease states, prophylactic pacing is recommended in asymptomatic patients due to the high risk of progression to complete heart block.[thecardiologyadvisor.com]
Death in Infancy
  • […] in Infancy Focus on Prolonged Repolarization 924 Sudden Cardiac Death in Kidney Diseases 934 Index 943 حقوق النشر[books.google.com]
Falling
  • So if you can “tomber amoureux” (fall in love), why not “tomber fou”? This notion of fall (“chute”) is more appropriate for expressing Creole experiences. So the narrator’s language is characterized by a literary diglossia.[potomitan.info]
Sleep Apnea
  • Apnea and Sudden Death 898 Sudden Cardiac Death in Athletes 911 Sudden Cardiac Death in Infancy Focus on Prolonged Repolarization 924 Sudden Cardiac Death in Kidney Diseases 934 Index 943 حقوق النشر[books.google.com]
Heart Disease
  • The majority of these new referrals are not for congenital heart disease.[books.google.com]
  • 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]
  • 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.[symptoma.com]
  • 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]
Heart Disease
  • The majority of these new referrals are not for congenital heart disease.[books.google.com]
  • 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]
  • 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.[symptoma.com]
  • 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]
Cyanosis
  • Most congenital heart disease will be evident in early life, presenting with cyanosis, heart murmur, congestive heart failure or shock.[books.google.com]
  • Examination Note whether the patient looks well or there are signs of inadequate cardiac output - for example, cold peripheries, peripheral cyanosis or features of congestive cardiac failure.[patient.info]
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]
  • While some patients exhibit no symptoms, others experience dyspnea, lightheadedness, dizziness, abdominal pain, and syncope.[symptoma.com]
  • 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]
  • 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]
  • 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]

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.

Prolonged PR Interval
  • 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.[symptoma.com]
  • PR interval, early repolarization pattern) 2280 (7.0) Total 3853 (11.8) RBBB, right bundle branch block; LVH, left ventricular hypertrophy; LBBB, left bundle branch block.[ 2 ] Classification of abnormalities of the athlete’s electrocardiogram Table[en.ecgpedia.org]
Third Degree Atrioventricular Block
  • Degree Atrioventricular Block (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[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]
T Wave Alternans
  • Wave Alternans Mechanisms and Implications for Prediction of Sudden Cardiac Death 394 Invasive Electrophysiologic Testing Role in Sudden Death Prediction 409 Provocative Testing in Inherited Arrhythmias 424 Novel Predictors of Sudden Cardiac Death 434[books.google.com]
  • L-type Ca2 channel mutations and T-wave alternans: a model study . Am J Physiol Heart Circ Physiol 2007; 293 : H3480–H3489. 87.[nature.com]

Treatment
  • 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]
  • 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]
  • The revolution in molecular medicine makes it imperative to integrate the classical and molecular approaches to drug development for the treatment of heart disease, the number one killer in North America and Europe.[books.google.com]
  • […] 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 and do not require treatment[en.cnki.com.cn]
  • Usually asymptomatic and treatment not required. Treat if symptomatic or in the context of inferior myocardial infarction. Mobitz type II : Conduction defect below AV node.[patient.info]

Prognosis
  • 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]
  • 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]
  • "Exercise-induced left bundle branch block: prevalence and prognosis". Arquivos Brasileiros De Cardiologia . 97 (1): 26–32. PMID 21552647 . Iscove NN, Yan XQ (1990).[wikidoc.org]
  • Prognosis Once appropriately paced, the prognosis for patients with symptomatic complete heart block depends on their underlying disease process.[thecardiologyadvisor.com]
  • The prevalence and prognosis of a Brugada-type electrocardiogram in a population of middle-aged Japanese-American men with follow-up of three decades . Am J Med Sci 2006; 331 : 25–29. 34. Van Norstrand DW , Valdivia CR , Tester DJ , et al.[nature.com]

Etiology
  • 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]
  • Etiology and pathology of bilateral bundle branch block in relation to complete heart block. Prog Cardiovasc Dis 1964;6:409-444. PMID 14153648 . External links [ edit ][en.wikipedia.org]
  • According to Ducimetiere: “There is now much evidence that the southern European diet and other lifestyle factors play a part and may modulate the effect of cholesterol and fat in the etiology of coronary heart disease.[epi.umn.edu]
  • Table 16.1 Etiology of complete heart block Isolated complete heart block—No associated structural heart disease • Congenital HB—Immune mediated • Congenital HB—nonimmune mediated • Cardiac manifestation of neuromuscular disease – Emery–Dreifuss muscular[thoracickey.com]

Epidemiology
  • The book spans topics such as aging changes in the cardiovascular system, risk factors and epidemiology for coronary artery disease, valvular heart disease, cardiomyopathies and heart failure, arrhythmias, cerebrovascular disease, and other miscellaneous[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]
  • Epidemiology It is impossible to give meaningful figures on incidence and prevalence. In most young people bradycardia is physiological and represents athletic training.[patient.info]
  • Association Scientific 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[nature.com]
Sex distribution
Age distribution

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]
  • […] 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]
  • 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]
  • These functional defects are called “primary electrical disease of the heart.” 3 , 4 , 5 , 6 The pathophysiological mechanisms underlying CCD are diverse, but the most frequent form of CCD is a degenerative form also called Lenegre-Lev disease 7 , 8 ([link.springer.com]
  • In summary, the biophysical findings therefore indicated a role of gain-of-function mutations in potassium channels in AF highlighting the pathophysiological role of shortened atrial action potentials.[nature.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]
  • This may not prevent tachycardias from occurring so you may also need to take anti-arrhythmic drugs. Some people may need to have an ICD fitted instead of a pacemaker. Medication alone does not help.[c-r-y.org.uk]
  • I was the first to propose that referees and handlers use gloves when handling boxers during fights to prevent the transmission of HIV infection.[einstein.yu.edu]
  • Prevention Appropriate treatment of any underlying cause - eg, hypothyroidism. Glycopyrrolate (antimuscarinic agent) is used in anaesthesia to prevent the bradycardia induced by cholinergic drugs such as the anticholinesterases.[patient.info]
  • Since every patient is different, it is important to undergo diagnosis and, if necessary, treatment, to prevent the progression of the condition.[vacardio.com]

References

Article

  1. Lev M, Kinare SG, Pick A. The pathogenesis of atrioventricular block in coronary disease. Circulation. 1970;42(3):409-25.
  2. Lev M, Cuadros H, Milton HP. Interruption of the atrioventricular bundle with congenital atrioventricular block. Circulation. 1971;43(5):703-10.
  3. 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.
  4. Schott JJ, Alshinawi C, Kyndt F, et al. Cardiac conduction defects associate with mutations in SCN5A. Nat Genet. 1999; 23(1):20–21.
  5. Baruteau AE, Probst V, Abriel H. Inherited progressive cardiac conduction disorders. Curr Opin Cardiol. 2015;30(1):33-9.
  6. Lev M. The pathology of complete atrioventricular block. Prog Cardiovasc Dis. 1964;6:317-26.
  7. 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.

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Last updated: 2018-06-22 09:38