Lev's disease, also known as Lenegre disease, is an idiopathic disease that can result in a complete heart block in patients with this condition. Impairment of the electrical conduction system of the heart may lead to fibrosis and calcification. This disease is considered to be age related, with increasing decline seen in elderly patients.
| Lev's disease | |
|---|---|
| Other names | Lenegre–Lev syndrome |
 | |
| pacemaker used for treatment of Lev's disease | |
| Specialty | Cardiology |
One form has been associated with mutations in the gene that encodes SCN5A.[1]
The use of electrocardiograms, especially in non-specialized settings like emergency rooms, may incidentally reveal a dysrhythmia that can confuse diagnosis, however serial ECGs will demonstrate an evolving conduction block arrhythmia characteristic of Lev’s disease, thus allowing for correct diagnosis.[2]
Presentation
editSigns and Symptoms
editAssociated conditions
editStokes–Adams attacks can be precipitated by this condition. These involve a temporary loss of consciousness resulting from marked slowing of the heart when the atrial impulse is no longer conducted to the ventricles. This should not be confused with the catastrophic loss of heartbeat seen with ventricular fibrillation or asystole.[citation needed]
History
editIn March 1954, researchers Richman and Wolff analyzed several patient cases using electrocardiograms and vectorcardiograms.[3] In terms of the different cardiovascular diagnostic tests available, electrocardiograms are the most widely used between physicians.[4] Many providers prefer the use of electrocardiograms since the process of interpreting vectorcardiograms are more complex and are more labor intensive as it requires more electrode placement on patients.[5] Electrocardiograms record and detect the electrical impulses generated by the heart.[5] By doing so, medical professionals are able to analyze the electrical activity of the heart and detect any irregularities that may impact the function of the heart.[5] The vectorcardiogram diagnostic test operates differently from electrocardiograms.[5] This vectorcardiogram method analyzes the transverse, sagittal, and frontal plane to measure the electrical activity of the heart.[5] This diagnostic test examines different factors such as rotations, contours, and the direction of the cardiac axis of the heart.[5] Although the use of electrocardiograms are commonly used in clinical settings by many providers, vectorcardiograms are able to be more precise due to it's ability to detect and identify the location of myocardial infarction, cardiac blockage, and hypertrophy.[5] Among these cases, patients exhibited a left bundle branch block that resembled a right bundle branch block.[3] Although the electrocardiograms showed a right bundle branch block, the vectorcardiograms detected a left bundle branch block.[3]
In March 1964, Jean Lenègre, published a paper discussing the pathology behind the gradual damage and scarring of the ventricular conduction system.[6]
Epidemiology
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Pathophysiology
editLev's disease operates similarly to other atrioventricular (AV) conduction disturbances. Unfortunately, the specific mechanisms of this condition are not yet fully clear. However, it is suspected that like other AV conduction disorders, Lev's disease can occur via two ways, acquired or congenital.[7] Individuals with congenital Lev's disease typically came from pregnancies with lupus erthematosus complications or transfer of SSA/Ro and SSB/LA antibodies.[8] In contrasat, there are many theories on the Lev's disease may be aquired by a patient, however, there is strong evidence of fibrosis of the conduction system leading to impairments of the conduction system.
Diagnosis
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Prevention
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Treatment and Management
editLev's disease is also known as Progressive cardiac conduction defect (PCCD)[9]
There are no studies that were conducted to determine what is the best treatment option for Lev's disease. However, this disease can be treated with a pacemaker[10][11]
See also
editReferences
edit- ^ 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. S2CID 7595466.
- ^ Carius, Brandon M.; Long, Brit; Schauer, Steve (May 2019). "Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department". The American Journal of Emergency Medicine. 37 (5): 1006.e1–1006.e4. doi:10.1016/j.ajem.2019.01.054. PMID 30723001.
- ^ a b c Richman, Justin L.; Wolff, Louis (1954). "Left bundle branch block masquerading as right bundle branch block". American Heart Journal. 47 (3): 383–393. doi:10.1016/0002-8703(54)90295-1.
- ^ Cook, David A.; Oh, So-Young; Pusic, Martin V. (2020-11-01). "Accuracy of Physicians' Electrocardiogram Interpretations: A Systematic Review and Meta-analysis". JAMA Internal Medicine. 180 (11): 1461. doi:10.1001/jamainternmed.2020.3989. ISSN 2168-6106. PMC 7522782. PMID 32986084.
- ^ a b c d e f g Jaros, Rene; Martinek, Radek; Danys, Lukas (2019-07-11). "Comparison of Different Electrocardiography with Vectorcardiography Transformations". Sensors. 19 (14): 3072. Bibcode:2019Senso..19.3072J. doi:10.3390/s19143072. ISSN 1424-8220. PMC 6678609. PMID 31336798.
- ^ Lenegre, Jean (1964). "Etiology and pathology of bilateral bundle branch block in relation to complete heart block". Progress in Cardiovascular Diseases. 6 (5): 409–444. doi:10.1016/S0033-0620(64)80001-3.
- ^ Wessels, Andy; McQuinn, Tim (2009), Lang, Florian (ed.), "Atrioventricular Conduction Disturbances", Encyclopedia of Molecular Mechanisms of Disease, Berlin, Heidelberg: Springer, pp. 179–181, doi:10.1007/978-3-540-29676-8_173, ISBN 978-3-540-29676-8, retrieved 2024-07-26
- ^ Wessels, Andy; McQuinn, Tim (2009), Lang, Florian (ed.), "Atrioventricular Conduction Disturbances", Encyclopedia of Molecular Mechanisms of Disease, Berlin, Heidelberg: Springer, pp. 179–181, doi:10.1007/978-3-540-29676-8_173#citeas, ISBN 978-3-540-29676-8, retrieved 2024-07-26
- ^ Schott, Jean-Jacques; Alshinawi, Connie; Kyndt, Florence; Probst, Vincent; Hoorntje, Theo M.; Hulsbeek, Miriam; Wilde, Arthur A. M.; Escande, Denis; Mannens, Marcel M. A. M.; Le Marec, Hervé (September 1999). "Cardiac conduction defects associate with mutations in SCN5A". Nature Genetics. 23 (1): 20–21. doi:10.1038/12618. ISSN 1546-1718. PMID 10471492.
- ^ Barra, SéRgio Nuno Craveiro; ProvidêNcia, Rui; Paiva, LuíS; Nascimento, José; Marques, AntóNio LeitãO (2012). "A Review on Advanced Atrioventricular Block in Young or Middle-Aged Adults". Pacing and Clinical Electrophysiology. 35 (11): 1395–1405. doi:10.1111/j.1540-8159.2012.03489.x. ISSN 0147-8389. PMID 22897386.
- ^ Carius, Brandon M.; Long, Brit; Schauer, Steve (2019-05-01). "Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department". The American Journal of Emergency Medicine. 37 (5): 1006.e1–1006.e4. doi:10.1016/j.ajem.2019.01.054. ISSN 0735-6757. PMID 30723001.