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Lev's disease

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158:. In terms of the different cardiovascular diagnostic tests available, electrocardiograms are the most widely used between physicians. 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. Electrocardiograms record and detect the electrical impulses generated by the heart. 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. However, vectorcardiogram diagnostic tests operate differently from electrocardiograms. This vectorcardiogram method analyzes the transverse, sagittal, and frontal planes to measure the electrical activity of the heart. This diagnostic test examines different factors such as rotations, contours, and the direction of the cardiac axis of the heart. Although the use of electrocardiograms are commonly used in clinical settings by many providers, vectorcardiograms are able to be more precise due to its ability to detect and identify the location of myocardial infarction, cardiac blockage, and hypertrophy. Among cases studied, patients exhibited a left bundle branch block that resembled a right bundle branch block. In comparison, however, although the electrocardiograms showed a right bundle branch block, the vectorcardiograms detected a left bundle branch block in the patient. 193:
the propagation of electrical currents through the heart. It has been seen that with age, the heart's autonomic movements begin to show rapid signs of decline, due to being governed by its ability to conduct signaling. Such issues occurring at the SA and AV nodes, will result in prorogation of ECG segments, because the pacemaker cells in these production centers are responsible for the rest of the signals traveling through the heart, and therefore the mechanical movements of cardiac muscle. In severe cases, like in Lev's disease, this can result in entire blockage of nodal electrical propagation.
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chest pain, dizziness, fatigue, syncope, shortness of breath. Physical examinations are also conducted to determine whether a patient has signs of bradycardia. One of the most important exams that can be conducted to help determine atrioventricular (AV) conduction block and bundle branch block is an electrocardiogram. Electrocardiograms are used primarily to measure how efficiently the heart is working. The function of electrocardiograms is detection of electrical signals in the heart. These electrical signals are recorded on a graph to help detect heart rhythm and heart attacks.
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SCN5a gene, which impacts the formation of Na+ channels, leaving them with myocardial conditions similar to those with Lev's disease. The study used these mice to gain a further understanding into the progression of such abnormalities, and how it can be applied to the similar impacts of fibrosis and calcification in human myocardial systems. Their study results found that in mice with this heterozygous mutation, with age, exhibited extensive fibrosis of cardiac tissue in comparison to mice without the mutation.
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adult. On the other hand, symptoms of Lev's disease may include bradycardia, defects in the cardiac conduction of the His-Purkinje system, widening of the QRS complex, and temporal potentially prolongation of the PR interval. However, individuals may also be asymptomatic, presenting with no symptoms. Thus, careful genetic screening and identification of patients with Lev's disease is important.
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Digoxin, beta blockers, calcium channel blockers, and anti-arrhythmic medications can slow down heart conduction in patients with pre-existing cardiac conditions. Since other cardiac conditions can be associated with Lev's disease, it is important to promote a heart healthy lifestyle. This includes adopting dietary and lifestyle changes that support and promote healthy heart health.
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Individuals with congenital Lev's disease typically come from pregnancies with lupus erthematosus complications or transfer of SSA/Ro and SSB/LA antibodies. In contrast, there are many theories on how Lev's disease may be acquired by a patient, however, there is strong evidence of fibrosis of the conduction system leading to impairments of the conduction system.
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to those for atrioventricular (AV) blocks. Both conditions involve the inability of the heart to properly conduct electrical signals; irregularity of heart beats and bradycardia. Treatment could involve implantation of a pacemaker to help restore and maintain a normal heart rhythm with ECG tests to ensure that the pacemaker is working properly.
74:, or an extremely slowed heart rate, in patients with this condition. It is thought that for certain patients, this impairment of heart's electrical conduction system is due to fibrosis and calcification of conduction cells. This disease is considered to be age related, with increasing decline seen in elderly patients. 192:
The heart is composed of two primary types of cell, contractile cells and specific cells that carry out conduction roles, including directing the actions of contractile cells. In the case of Lev's disease, calcification or fibrosis of these cells will impact their ability to work optimally, hindering
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is another condition that can be precipitated by Lev's disease. This condition is the interrupted conduction of electrical impulses along the Bundle of His or one of the bundle branches. As the Bundle of His divides into the right and left bundle branches, a block of the electrical impulse may result
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Lev's disease continues to be an active area of research. Currently, there is limited case reports, studies, and trials revolving around the treatment for Lev's Disease. There is no case study that gives a definitive treatment option. However, its treatment and management can be approached similarly
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Therefore, differential diagnoses can be conducted to help narrow down the list of possible diseases and help providers determine whether a patient may or may not have this condition. These approaches include using clinical evaluation to assess patient's past medical history for any symptoms such as
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In November 1964, Maurice Lev published a paper with similar findings as Lenègre where he saw the degenerative processes of the ventricular conduction system associated with calcification in older patient populations. Lev focused primarily on the anatomical process leading to atrioventricular blocks
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Over the years, many clinical studies have been conducted to give insight on the congenital acquirement of Lev's Disease, particularly to answer the question of if there is a genetic component that puts patients at a predisposed risk. A mouse model studied mice with a heterogenous mutation to their
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Researchers do understand that Lev's disease can be presented in two primary forms: acquired and congenital. Individuals who have acquired Lev's disease presents signs and symptoms later in life and are often linked to the use of medication, medical conditions, surgical procedures, or environmental
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Diagnosis of Lev's disease can be difficult due to the limited supporting literature. However, given that the disease shares similarities to conditions characterized by atrioventricular (AV) conduction blocks and bundle branch blocks, differential diagnostic approaches can involve considerations of
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A preventive strategy can involve regular electrocardiogram tests, especially if the individual has a genetic family history of cardiac conditions such as Lev's disease. Additionally, the use of certain medications can impact the conduction of the heart. Studies have shown that medications such as
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Lev'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.
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In a genetic epidemiological study published in 2012 focusing on western France, there were 6667 patients implanted with pacemakers for PCCD between the years of 1995 and 2005. The frequency of PCCD in different areas of western France was concluded to be 0.21% in a major city and up to 2.28% in
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Lev's disease may present with various signs and symptoms; however, these may overlap with similar conditions such as atrioventricular (AV) blocks. Signs of Lev's disease may include syncope, shortness of breath, dizziness, heart failure, and sudden cardiac death. Signs may begin to appear as an
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factors. In cases of congenital Lev's disease, it is present from birth and is commonly caused by genetic or developmental factors. Depending if the patient has acquired or congenital Lev's disease, the prevention strategies may differ and vary to address the needs associated with each form.
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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
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Lev's disease remains an area of ongoing research and the specific underlying mechanisms of this disease state are not fully understood. As a result, effective prevention methods are currently limited.
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that can confuse diagnosis, however serial ECGs will demonstrate an evolving conduction block arrhythmia characteristic of Lev's disease, thus allowing for correct diagnosis.
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may be a progression of Lev's disease, in which the electrical signal transmission from the heart's atria to the ventricles is impaired due to an extension of the fibrosis.
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One study published in 1998 followed 855 men born in 1913, and among the group the prevalence of bundle branch block was 17% when the study population was at age 80 years.
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While there is limited data directly addressing Lev's disease and its prevalence, there are also some statistics on the associated diseases precipitated by Lev's disease.
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Schott JJ, Alshinawi C, Kyndt F, Probst V, Hoorntje TM, Hulsbeek M, et al. (September 1999). "Cardiac conduction defects associate with mutations in SCN5A".
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specific parishes. Five large families were also identified to be affected by PCCD, contributing to the understanding that the disease has a genetic factor.
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Carius BM, Long B, Schauer S (May 2019). "Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department".
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In March 1964, Jean Lenègre published a paper discussing the pathology behind the gradual damage and scarring of the ventricular conduction system.
438:"Whole exome sequencing identified a pathogenic nonsense mutation in LMNA in a family with a progressive cardiac conduction defect: A case report" 932: 601: 552: 146:
may also result be a result of Lev's disease. During a SA block, the electrical impulse formed at the SA node is delayed or not conducted.
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Lenegre J (March 1964). "Etiology and pathology of bilateral bundle branch block in relation to complete heart block".
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In March 1954, researchers Richman and Wolff analyzed several patient cases using electrocardiograms and
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Richman JL, Wolff L (March 1954). "Left bundle branch block masquerading as right bundle branch block".
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255: 1505: 1324: 1272: 294: 70:, also known as Lenegre disease, is an idiopathic disease that can result in a 1151:
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in patients and was able to build off the research conducted by Lenègre.
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Retrieved 320: 311: 286: 282: 244: 236:Case studies 230: 221: 217: 213: 204: 200: 191: 187: 179: 176: 172: 169:Epidemiology 163: 160: 153: 142: 127: 121: 106: 97: 89:Presentation 76: 67: 66: 35: 32: 15: 1110:Circulation 871:Circulation 346:Circulation 256:Heart block 83:dysrhythmia 77:The use of 29:Other names 1501:DiseasesDB 1290:2024-07-28 1277:StatPearls 1258:2024-07-30 609:2024-07-28 560:2024-07-30 326:2024-07-30 262:References 210:Prevention 61:Cardiology 1424:1546-1718 1334:0033-0620 1226:1015-9584 1177:0002-8703 1130:0009-7322 1021:2152-5250 974:0160-9289 891:0009-7322 844:1468-201X 462:1791-2997 415:1347-4820 366:0009-7322 197:Diagnosis 57:Specialty 1522:Category 1432:10471492 1377:10471492 1342:34922952 1318:: 8–15. 1285:29939649 1234:36167659 1138:15809371 1088:26835096 1039:37163425 1030:10389818 852:22717692 805:14237429 770:31336798 698:32986084 649:13124253 518:14153648 480:32323820 423:23018635 374:16567565 303:30723001 250:See also 117:asystole 1495:C566873 1385:7595466 1079:4711507 982:8348766 899:9832497 761:6678609 738:Bibcode 730:Sensors 689:7522782 471:7185278 150:History 1484:113900 1430:  1422:  1383:  1375:  1340:  1332:  1283:  1232:  1224:  1185:910682 1183:  1175:  1136:  1128:  1086:  1076:  1037:  1027:  1019:  980:  972:  931:  897:  889:  850:  842:  803:  768:  758:  696:  686:  647:  600:  551:  516:  478:  468:  460:  421:  413:  372:  364:  301:  1506:33970 1473:426.0 1381:S2CID 136:or a 132:in a 1490:MeSH 1479:OMIM 1468:9-CM 1428:PMID 1420:ISSN 1373:PMID 1338:PMID 1330:ISSN 1281:PMID 1230:PMID 1222:ISSN 1181:PMID 1173:ISSN 1134:PMID 1126:ISSN 1084:PMID 1035:PMID 1017:ISSN 978:PMID 970:ISSN 929:ISBN 895:PMID 887:ISSN 848:PMID 840:ISSN 801:PMID 766:PMID 694:PMID 645:PMID 598:ISBN 549:ISBN 514:PMID 476:PMID 458:ISSN 419:PMID 411:ISSN 370:PMID 362:ISSN 299:PMID 1464:ICD 1412:doi 1365:doi 1320:doi 1212:doi 1165:doi 1118:doi 1114:111 1074:PMC 1066:doi 1025:PMC 1009:doi 962:doi 921:doi 879:doi 832:doi 793:doi 756:PMC 746:doi 684:PMC 676:doi 672:180 637:doi 590:doi 541:doi 506:doi 466:PMC 450:doi 401:doi 354:doi 350:113 291:doi 115:or 1524:: 1504:: 1493:: 1482:: 1471:: 1426:. 1418:. 1408:23 1406:. 1402:. 1379:. 1371:. 1361:23 1359:. 1336:. 1328:. 1316:70 1314:. 1310:. 1298:^ 1275:, 1250:, 1228:. 1220:. 1208:46 1206:. 1202:. 1179:. 1171:. 1161:94 1159:. 1155:. 1132:. 1124:. 1112:. 1108:. 1096:^ 1082:. 1072:. 1060:. 1056:. 1033:. 1023:. 1015:. 1005:14 1003:. 999:. 976:. 968:. 958:16 956:. 952:. 927:. 907:^ 893:. 885:. 875:98 873:. 869:. 846:. 838:. 828:98 826:. 822:. 799:. 789:37 787:. 764:. 754:. 744:. 734:19 732:. 728:. 706:^ 692:. 682:. 670:. 666:. 643:. 633:47 631:. 617:^ 596:, 584:, 568:^ 547:, 535:, 512:. 500:. 488:^ 474:. 464:. 456:. 446:21 444:. 440:. 417:. 409:. 397:77 395:. 391:. 368:. 360:. 348:. 344:. 319:. 297:. 287:37 285:. 269:^ 119:. 1466:- 1456:D 1434:. 1414:: 1387:. 1367:: 1344:. 1322:: 1236:. 1214:: 1187:. 1167:: 1140:. 1120:: 1090:. 1068:: 1062:4 1041:. 1011:: 984:. 964:: 937:. 923:: 901:. 881:: 854:. 834:: 807:. 795:: 772:. 748:: 740:: 700:. 678:: 651:. 639:: 592:: 543:: 520:. 508:: 502:6 482:. 452:: 425:. 403:: 376:. 356:: 329:. 305:. 293::

Index


Specialty
complete heart block
electrocardiograms
dysrhythmia
Stokes–Adams attacks
ventricular fibrillation
asystole
Atrioventricular (AV) block
Bundle Branch Block (BBB)
Right Bundle Branch Block
Left Bundle Branch Block
Sinoatrial (SA) block
vectorcardiograms
Heart block




doi
10.1016/j.ajem.2019.01.054
PMID
30723001
"Familial progressive cardiac conduction defect - About the Disease - Genetic and Rare Diseases Information Center"
"Contemporary Definitions and Classification of the Cardiomyopathies: An American Heart 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 and Prevention"
doi
10.1161/CIRCULATIONAHA.106.174287
ISSN
0009-7322
PMID

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