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Increased QRS voltage

MedGen UID:
909971
Concept ID:
C1112650
Finding
HPO: HP:0025075

Definition

Elevation of the voltage (height) of the QRS complex. There are several criteria in use, but the most common is the Sokolov-Lyon criterion (S wave depth in V1 + tallest R wave height in V5-V6 greater than 35 mm). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased QRS voltage

Conditions with this feature

Danon disease
MedGen UID:
209235
Concept ID:
C0878677
Disease or Syndrome
Danon disease is a multisystem condition with predominant involvement of the heart, skeletal muscles, and retina, with overlying cognitive dysfunction. Males are typically more severely affected than females. Males usually present with childhood onset concentric hypertrophic cardiomyopathy that is progressive and often requires heart transplantation. Rarely, hypertrophic cardiomyopathy can evolve to resemble dilated cardiomyopathy. Most affected males also have cardiac conduction abnormalities. Skeletal muscle weakness may lead to delayed acquisition of motor milestones. Learning disability and intellectual disability, most often in the mild range, are common. Additionally, affected males can develop retinopathy with subsequent visual impairment. The clinical features in females are broader and more variable. Females are more likely to have dilated cardiomyopathy, with a smaller proportion requiring heart transplantation compared to affected males. Cardiac conduction abnormalities, skeletal muscle weakness, mild cognitive impairment, and pigmentary retinopathy are variably seen in affected females.
Myofibrillar myopathy 10
MedGen UID:
1769385
Concept ID:
C5436656
Disease or Syndrome
Myofibrillar myopathy-10 (MFM10) is an autosomal recessive structural muscle disorder characterized by onset of muscle pain, cramping, and exercise fatigue in the first or second decades of life. Some patients have mild contractures of the large joints apparent in early childhood. Affected individuals have a characteristic appearance of a thick neck and prominent shoulder girdle with anteverted shoulders and a tendency toward kyphosis. There is no apparent muscle weakness, but some affected individuals show progressive muscle rigidity leading to limited mobility. There is variable cardiac involvement, ranging from chest pain with left ventricular hypertrophy to subclinical signs such as abnormal EKG or elevated cardiac enzymes. Skeletal muscle biopsy shows structural abnormalities with myofibrillar disorganization and accumulation of autophagocytic vacuoles (summary by Hedberg-Oldfors et al., 2020). For a general phenotypic description and a discussion of genetic heterogeneity of myofibrillar myopathy, see MFM1 (601419).

Professional guidelines

PubMed

Bacharova L
J Electrocardiol 2014 Sep-Oct;47(5):625-9. Epub 2014 May 27 doi: 10.1016/j.jelectrocard.2014.05.006. PMID: 24973139

Recent clinical studies

Etiology

Vecchiato M, Baioccato V, Adami PE, Quinto G, Foccardi G, Slanzi G, Battista F, Neunhaeuserer D, Ermolao A
Scand J Med Sci Sports 2022 Nov;32(11):1581-1591. Epub 2022 Sep 15 doi: 10.1111/sms.14232. PMID: 36086882Free PMC Article
Bacharova L
J Electrocardiol 2022 Jul-Aug;73:153-156. Epub 2022 Jun 14 doi: 10.1016/j.jelectrocard.2022.06.002. PMID: 35718553
Cichoż-Lach H, Tomaszewski M, Kowalik A, Lis E, Tomaszewski A, Lach T, Boczkowska S, Celiński K
Adv Clin Exp Med 2015 Jul-Aug;24(4):615-22. doi: 10.17219/acem/28681. PMID: 26469105
Noseworthy PA, Weiner R, Kim J, Keelara V, Wang F, Berkstresser B, Wood MJ, Wang TJ, Picard MH, Hutter AM Jr, Newton-Cheh C, Baggish AL
Circ Arrhythm Electrophysiol 2011 Aug;4(4):432-40. Epub 2011 May 4 doi: 10.1161/CIRCEP.111.962852. PMID: 21543642Free PMC Article
Bacharova L, Estes EH
J Electrocardiol 2009 May-Jun;42(3):228-32. doi: 10.1016/j.jelectrocard.2009.03.005. PMID: 19398051

Diagnosis

Bacharova L
J Electrocardiol 2022 Jul-Aug;73:153-156. Epub 2022 Jun 14 doi: 10.1016/j.jelectrocard.2022.06.002. PMID: 35718553
Bacharova L, Estes EH
J Electrocardiol 2017 Nov-Dec;50(6):906-908. Epub 2017 Jun 7 doi: 10.1016/j.jelectrocard.2017.06.006. PMID: 28651797
Cichoż-Lach H, Tomaszewski M, Kowalik A, Lis E, Tomaszewski A, Lach T, Boczkowska S, Celiński K
Adv Clin Exp Med 2015 Jul-Aug;24(4):615-22. doi: 10.17219/acem/28681. PMID: 26469105
Chakko S, Myerburg RJ
Clin Cardiol 1995 Feb;18(2):67-72. doi: 10.1002/clc.4960180206. PMID: 7720292
Gruber JE
J Emerg Med 1985;3(1):37-9. doi: 10.1016/0736-4679(85)90218-5. PMID: 2936795

Therapy

Chakko S, Sepulveda S, Kessler KM, Sotomayor MC, Mash DC, Prineas RJ, Myerburg RJ
Am J Cardiol 1994 Oct 1;74(7):710-3. doi: 10.1016/0002-9149(94)90315-8. PMID: 7942531

Prognosis

Bacharova L, Estes EH
J Electrocardiol 2017 Nov-Dec;50(6):906-908. Epub 2017 Jun 7 doi: 10.1016/j.jelectrocard.2017.06.006. PMID: 28651797
Cichoż-Lach H, Tomaszewski M, Kowalik A, Lis E, Tomaszewski A, Lach T, Boczkowska S, Celiński K
Adv Clin Exp Med 2015 Jul-Aug;24(4):615-22. doi: 10.17219/acem/28681. PMID: 26469105
Bacharova L, Szathmary V, Kovalcik M, Mateasik A
J Electrocardiol 2010 May-Jun;43(3):200-8. Epub 2009 Aug 26 doi: 10.1016/j.jelectrocard.2009.07.014. PMID: 19709670
Salerno DM, Asinger RW, Elsperger J, Erlien D, Hodges M
J Electrocardiol 1988 Nov;21(4):303-12. doi: 10.1016/0022-0736(88)90106-9. PMID: 3241141
Rabkin SW
Drugs 1984 Oct;28 Suppl 1:28-45. doi: 10.2165/00003495-198400281-00004. PMID: 6209083

Clinical prediction guides

Bacharova L, Estes EH
J Electrocardiol 2017 Nov-Dec;50(6):906-908. Epub 2017 Jun 7 doi: 10.1016/j.jelectrocard.2017.06.006. PMID: 28651797
Cichoż-Lach H, Tomaszewski M, Kowalik A, Lis E, Tomaszewski A, Lach T, Boczkowska S, Celiński K
Adv Clin Exp Med 2015 Jul-Aug;24(4):615-22. doi: 10.17219/acem/28681. PMID: 26469105
Bacharova L
J Electrocardiol 2014 Sep-Oct;47(5):625-9. Epub 2014 May 27 doi: 10.1016/j.jelectrocard.2014.05.006. PMID: 24973139
Bacharova L, Szathmary V, Kovalcik M, Mateasik A
J Electrocardiol 2010 May-Jun;43(3):200-8. Epub 2009 Aug 26 doi: 10.1016/j.jelectrocard.2009.07.014. PMID: 19709670
Gruber JE
J Emerg Med 1985;3(1):37-9. doi: 10.1016/0736-4679(85)90218-5. PMID: 2936795

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