Lev's disease
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Synonyms and keywords: Lenègre-Lev disease, Lenègre’s disease, Maladie de Lenègre, Acquired complete heart block
Overview
Lev's disease is an acquired complete heart block due to idiopathic fibrosis and calcification of the electrical conduction system of the heart. Lev's disease is most commonly seen in the elderly, and is often described as senile degeneration of the conduction system.
One form has been associated with SCN5A.[1]
Historical Perspective
- In 1954, Lenègre published the condition in French, hence, it is also referred to as Lenègre’s disease or Maladie de Lenègre in French, after his name.[2]
- In 1964, it was described independently by the two researchers, Jean Lenègre and Maurice Lev in English, but the condition is generally called after Lev.[3][4][5][6]
Pathophysiology
- Fibrous transformation progressive and slow, of degenerative origin, of the two branches of the bundle of His, resulting in progressive conductional disorders:[7][8]
- block of branch with or without hemibloc of the opposite side
- then complete, paroxysmal then permanent block of auriculoventricular (disease of Adams-Stokes).
Associated Conditions
Stokes-Adams attacks can be precipitated by this condition. These involve a temporary loss of consciousness due to ventricular fibrillation or asystole.[9]
Related Chapters
References
- ↑ Schott JJ, Alshinawi C, Kyndt F; et al. (1999). "Cardiac conduction defects associate with mutations in SCN5A". Nat. Genet. 23 (1): 20–1. doi:10.1038/12618. PMID 10471492.
- ↑ LENEGRE J (1964). "ETIOLOGY AND PATHOLOGY OF BILATERAL BUNDLE BRANCH BLOCK IN RELATION TO COMPLETE HEART BLOCK". Prog Cardiovasc Dis. 6: 409–44. doi:10.1016/s0033-0620(64)80001-3. PMID 14153648.
- ↑ Lev M. Anatomic basis for atrioventricular block. Am J Med 1964;37:742-8. PMID 14237429.
- ↑ Lenegre J. Etiology and pathology of bilateral bundle branch block in relation to complete heart block. Prog Cardiovasc Dis 1964;6:409-444. PMID 14153648.
- ↑ LEV M (1964). "ANATOMIC BASIS FOR ATRIOVENTRICULAR BLOCK". Am J Med. 37: 742–8. doi:10.1016/0002-9343(64)90022-1. PMID 14237429.
- ↑ https://annals.org/aim/article-abstract/696353/lev-len-gre-diseases
- ↑ https://litfl.com/lenegre-lev-disease/
- ↑ https://academic.oup.com/europace/article/7/2/122/557245
- ↑ Carius BM, Long B, Schauer S (2019). "Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department". Am J Emerg Med. 37 (5): 1006.e1–1006.e4. doi:10.1016/j.ajem.2019.01.054. PMID 30723001.