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Left anterior fascicular block

MedGen UID:
75547
Concept ID:
C0264912
Disease or Syndrome
Synonym: Left anterior hemiblock
SNOMED CT: LAFB - Left anterior fascicular block (37760005); Left anterior hemiblock (37760005); Left anterior fascicular block (37760005)
 
HPO: HP:0011711

Definition

Conduction block in the anterior division of the left bundle branch of the bundle of His. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLeft anterior fascicular block

Conditions with this feature

Emery-Dreifuss muscular dystrophy 2, autosomal dominant
MedGen UID:
98048
Concept ID:
C0410190
Disease or Syndrome
Emery-Dreifuss muscular dystrophy (EDMD) is characterized by the clinical triad of: joint contractures that begin in early childhood; slowly progressive muscle weakness and wasting initially in a humero-peroneal distribution that later extends to the scapular and pelvic girdle muscles; and cardiac involvement that may manifest as palpitations, presyncope and syncope, poor exercise tolerance, and congestive heart failure along with variable cardiac rhythm disturbances. Age of onset, severity, and progression of muscle and cardiac involvement demonstrate both inter- and intrafamilial variability. Clinical variability ranges from early onset with severe presentation in childhood to late onset with slow progression in adulthood. In general, joint contractures appear during the first two decades, followed by muscle weakness and wasting. Cardiac involvement usually occurs after the second decade and respiratory function may be impaired in some individuals.
Progressive familial heart block, type 1A
MedGen UID:
406301
Concept ID:
C1879286
Disease or Syndrome
Progressive familial heart block type I (PFHBI, PFHB1) is an autosomal dominant cardiac bundle branch disorder that may progress to complete heart block (Brink and Torrington, 1977; van der Merwe et al., 1986; van der Merwe et al., 1988). It is defined on electrocardiogram by evidence of bundle branch disease, i.e., right bundle branch block, left anterior or posterior hemiblock, or complete heart block, with broad QRS complexes. Progression has been shown from a normal electrocardiogram to right bundle branch block and from the latter to complete heart block. These electrocardiographic features differentiate PFHB type I from progressive familial heart block type II (PFHBII, PFHB2; 140400), in which the onset of complete heart block is associated with narrow complexes. Electrocardiographically the changes represent, respectively, bundle branch disease (PFHB1) and atrioventricular nodal disease with an atrioventricular block and an idionodal escape rhythm (PFHB2). PFHBI is manifested symptomatically when complete heart block supervenes, either with dyspnea, syncopal episodes, or sudden death. Treatment, which is best managed by regular electrocardiographic follow-up, is by the timely implantation of a pacemaker (Brink et al., 1995). Genetic Heterogeneity of Progressive Familial Heart Block Type I Progressive familial heart block type IB (PFHB1B; 604559) is caused by mutation in the TRPM4 gene (606936) on chromosome 19q13.32.
Progressive familial heart block type IB
MedGen UID:
370220
Concept ID:
C1970298
Disease or Syndrome
Most cases of heart block are not genetic and are not considered progressive familial heart block. The most common cause of heart block is fibrosis of the heart, which occurs as a normal process of aging. Other causes of heart block can include the use of certain medications or an infection of the heart tissue.\n\nProgressive familial heart block can be divided into type I and type II, with type I being further divided into types IA and IB. These types differ in where in the heart signaling is interrupted and the genetic cause. In types IA and IB, the heart block originates in the bundle branch, and in type II, the heart block originates in the atrioventricular node. The different types of progressive familial heart block have similar signs and symptoms.\n\nHeart block occurs when the electrical signaling is obstructed anywhere from the atria to the ventricles. In people with progressive familial heart block, the condition worsens over time: early in the disorder, the electrical signals are partially blocked, but the block eventually becomes complete, preventing any signals from passing through the heart. Partial heart block causes a slow or irregular heartbeat (bradycardia or arrhythmia, respectively), and can lead to the buildup of scar tissue (fibrosis) in the cells that carry electrical impulses. Fibrosis contributes to the development of complete heart block, resulting in uncoordinated electrical signaling between the atria and the ventricles and inefficient pumping of blood in the heart. Complete heart block can cause a sensation of fluttering or pounding in the chest (palpitations), shortness of breath, fainting (syncope), or sudden cardiac arrest and death.\n\nProgressive familial heart block is a genetic condition that alters the normal beating of the heart. A normal heartbeat is controlled by electrical signals that move through the heart in a highly coordinated way. These signals begin in a specialized cluster of cells called the sinoatrial node (the heart's natural pacemaker) located in the heart's upper chambers (the atria). From there, a group of cells called the atrioventricular node carries the electrical signals to another cluster of cells called the bundle of His. This bundle separates into multiple thin spindles called bundle branches, which carry electrical signals into the heart's lower chambers (the ventricles). Electrical impulses move from the sinoatrial node down to the bundle branches, stimulating a normal heartbeat in which the ventricles contract slightly later than the atria.
Hypertrophic cardiomyopathy 11
MedGen UID:
436962
Concept ID:
C2677506
Disease or Syndrome
An autosomal dominant subtype of familial hypertrophic cardiomyopathy caused by mutation(s) in the ACTC1 gene, encoding actin, alpha cardiac muscle 1.
Hypertrophic cardiomyopathy 13
MedGen UID:
442487
Concept ID:
C2750472
Disease or Syndrome
Any hypertrophic cardiomyopathy in which the cause of the disease is a mutation in the TNNC1 gene.
Atrial conduction disease
MedGen UID:
863722
Concept ID:
C4015285
Disease or Syndrome
A rare genetic cardiac disease characterized by variably expressed atrial tachyarrhythmia (such as atrial flutter, paroxysmal or chronic atrial fibrillation, ectopic atrial tachycardia, or multifocal atrial tachycardia), infra-Hisian conduction system disease, and vulnerability to dilated cardiomyopathy. Age of onset ranges between childhood and adulthood.
Hypertrophic cardiomyopathy 26
MedGen UID:
934716
Concept ID:
C4310749
Disease or Syndrome
Familial cardiomyopathy caused by mutation in the FLNC gene has been described as hypertrophic, restrictive, dilated, or arrhythmogenic right ventricular cardiomyopathy. Affected individuals, especially those with dilated cardiomyopathy, are at risk for arrhythmias and sudden death. Arrhythmias without cardiomyopathy, and left ventricular noncompaction, have also been reported (Ortiz-Genga et al., 2016; Verdonschot et al., 2020).

Professional guidelines

PubMed

Bacharova L, Chevalier P, Gorenek B, Jons C, Li YG, Locati ET, Maanja M, Pérez-Riera AR, Platonov PG, Ribeiro ALP, Schocken D, Soliman EZ, Svehlikova J, Tereshchenko LG, Ugander M, Varma N, Elena Z, Ikeda T
Ann Noninvasive Electrocardiol 2024 Jan;29(1):e13097. Epub 2023 Nov 24 doi: 10.1111/anec.13097. PMID: 37997698Free PMC Article
Nyholm BC, Ghouse J, Lee CJ, Rasmussen PV, Pietersen A, Hansen SM, Torp-Pedersen C, Køber L, Haunsø S, Olesen MS, Svendsen JH, Graff C, Holst AG, Nielsen JB, Skov MW
Heart Rhythm 2022 Feb;19(2):252-259. Epub 2021 Oct 19 doi: 10.1016/j.hrthm.2021.09.041. PMID: 34673253
Kireyev D, Arkhipov MV, Zador ST, Paris JA, Boden WE
Ann Noninvasive Electrocardiol 2010 Apr;15(2):175-80. doi: 10.1111/j.1542-474X.2010.00359.x. PMID: 20522059Free PMC Article

Recent clinical studies

Etiology

Liu P, Wang Y, Zhang X, Zhang Z, Zhao N, Ou W, Wang G, Yang X, Li M, Zhang Y, Yang X, Wu S
JAMA Netw Open 2023 Nov 1;6(11):e2342831. doi: 10.1001/jamanetworkopen.2023.42831. PMID: 37955899Free PMC Article
Frimodt-Møller EK, Soliman EZ, Kizer JR, Vittinghoff E, Psaty BM, Biering-Sørensen T, Gottdiener JS, Marcus GM
Eur Heart J 2023 Mar 21;44(12):1058-1066. doi: 10.1093/eurheartj/ehac799. PMID: 36660815Free PMC Article
Nikus K, Birnbaum Y, Fiol-Sala M, Rankinen J, de Luna AB
Curr Cardiol Rev 2021;17(1):41-49. doi: 10.2174/1573403X16666200702121937. PMID: 32614749Free PMC Article
Chenniappan M, Sankar RU, Saravanan K, Karthikeyan
J Assoc Physicians India 2013 Sep;61(9):650-4. PMID: 24772703
Aronow WS
Compr Ther 1992 Nov;18(11):11-6. PMID: 1478052

Diagnosis

Liu P, Wang Y, Zhang X, Zhang Z, Zhao N, Ou W, Wang G, Yang X, Li M, Zhang Y, Yang X, Wu S
JAMA Netw Open 2023 Nov 1;6(11):e2342831. doi: 10.1001/jamanetworkopen.2023.42831. PMID: 37955899Free PMC Article
Nikus K, Birnbaum Y, Fiol-Sala M, Rankinen J, de Luna AB
Curr Cardiol Rev 2021;17(1):41-49. doi: 10.2174/1573403X16666200702121937. PMID: 32614749Free PMC Article
Chenniappan M, Sankar RU, Saravanan K, Karthikeyan
J Assoc Physicians India 2013 Sep;61(9):650-4. PMID: 24772703
Strauss DG, Selvester RH, Wagner GS
Am J Cardiol 2011 Mar 15;107(6):927-34. doi: 10.1016/j.amjcard.2010.11.010. PMID: 21376930
Aronow WS
Compr Ther 1992 Nov;18(11):11-6. PMID: 1478052

Therapy

Neira V, Enriquez A, Sheldon R, Hanson MG, Maxey C, Baranchuk A
Heart Rhythm 2023 Jan;20(1):31-36. Epub 2022 Sep 29 doi: 10.1016/j.hrthm.2022.09.018. PMID: 36184061
Rankinen J, Haataja P, Lyytikäinen LP, Huhtala H, Lehtimäki T, Kähönen M, Eskola M, Pérez-Riera AR, Jula A, Rissanen H, Nikus K, Hernesniemi J
Ann Noninvasive Electrocardiol 2021 Jan;26(1):e12788. Epub 2020 Aug 17 doi: 10.1111/anec.12788. PMID: 32804416Free PMC Article
Soe KK, Lee MY
Am J Case Rep 2019 Dec 27;20:1949-1955. doi: 10.12659/AJCR.919833. PMID: 31879415Free PMC Article
Cardoso R, Garcia D, Fernandes G, He LI, Lichtenberger P, Viles-Gonzalez J, Coffey JO, Mitrani RD
J Cardiovasc Electrophysiol 2016 Feb;27(2):161-9. Epub 2015 Nov 6 doi: 10.1111/jce.12845. PMID: 26412204
Strauss DG, Selvester RH, Wagner GS
Am J Cardiol 2011 Mar 15;107(6):927-34. doi: 10.1016/j.amjcard.2010.11.010. PMID: 21376930

Prognosis

Nikus K, Birnbaum Y, Fiol-Sala M, Rankinen J, de Luna AB
Curr Cardiol Rev 2021;17(1):41-49. doi: 10.2174/1573403X16666200702121937. PMID: 32614749Free PMC Article
Cardoso R, Garcia D, Fernandes G, He LI, Lichtenberger P, Viles-Gonzalez J, Coffey JO, Mitrani RD
J Cardiovasc Electrophysiol 2016 Feb;27(2):161-9. Epub 2015 Nov 6 doi: 10.1111/jce.12845. PMID: 26412204
Chenniappan M, Sankar RU, Saravanan K, Karthikeyan
J Assoc Physicians India 2013 Sep;61(9):650-4. PMID: 24772703
Elizari MV, Baranchuk A, Chiale PA
Expert Rev Cardiovasc Ther 2013 Jan;11(1):69-75. doi: 10.1586/erc.12.142. PMID: 23259447
Aronow WS
Compr Ther 1992 Nov;18(11):11-6. PMID: 1478052

Clinical prediction guides

Lakshman H, Banthiya S, Ahmed A, Shah D
Pacing Clin Electrophysiol 2023 Oct;46(10):1182-1185. Epub 2023 Aug 31 doi: 10.1111/pace.14808. PMID: 37650470
Gao Y, Xia L, Gong YL, Zheng DC
J Zhejiang Univ Sci B 2018 Jan.;19(1):49-56. doi: 10.1631/jzus.B1700029. PMID: 29308607Free PMC Article
Cardoso R, Garcia D, Fernandes G, He LI, Lichtenberger P, Viles-Gonzalez J, Coffey JO, Mitrani RD
J Cardiovasc Electrophysiol 2016 Feb;27(2):161-9. Epub 2015 Nov 6 doi: 10.1111/jce.12845. PMID: 26412204
Chenniappan M, Sankar RU, Saravanan K, Karthikeyan
J Assoc Physicians India 2013 Sep;61(9):650-4. PMID: 24772703
Aronow WS
Compr Ther 1992 Nov;18(11):11-6. PMID: 1478052

Recent systematic reviews

Mahajan S, Gupta R, Malik AH, Mahajan P, Aedma SK, Aronow WS, Mehta SS, Lakkireddy DR
J Cardiovasc Electrophysiol 2021 May;32(5):1411-1420. Epub 2021 Mar 17 doi: 10.1111/jce.14986. PMID: 33682218
Rojas LZ, Glisic M, Pletsch-Borba L, Echeverría LE, Bramer WM, Bano A, Stringa N, Zaciragic A, Kraja B, Asllanaj E, Chowdhury R, Morillo CA, Rueda-Ochoa OL, Franco OH, Muka T
PLoS Negl Trop Dis 2018 Jun;12(6):e0006567. Epub 2018 Jun 13 doi: 10.1371/journal.pntd.0006567. PMID: 29897909Free PMC Article
Kireyev D, Arkhipov MV, Zador ST, Paris JA, Boden WE
Ann Noninvasive Electrocardiol 2010 Apr;15(2):175-80. doi: 10.1111/j.1542-474X.2010.00359.x. PMID: 20522059Free PMC Article

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