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Right axis deviation

MedGen UID:
534422
Concept ID:
C0232296
Finding
Synonyms: Electrical right axis deviation; RAD; RAD - Right axis deviation
SNOMED CT: Right axis deviation (47665007); RAD - Right axis deviation (47665007)
 
HPO: HP:0033567

Definition

A kind of abnormal ventricular axis in the EKG whereby the QRS axis falls between +90 degrees and 180 degrees, or beyond +100 degrees if the adult range is used. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRight axis deviation

Conditions with this feature

Glycogen storage disease, type II
MedGen UID:
5340
Concept ID:
C0017921
Disease or Syndrome
Pompe disease is classified by age of onset, organ involvement, severity, and rate of progression. Infantile-onset Pompe disease (IOPD; individuals with onset before age 12 months with cardiomyopathy) may be apparent in utero but more typically onset is at the median age of four months with hypotonia, generalized muscle weakness, feeding difficulties, failure to thrive, respiratory distress, and hypertrophic cardiomyopathy. Without treatment by enzyme replacement therapy (ERT), IOPD commonly results in death by age two years from progressive left ventricular outflow obstruction and respiratory insufficiency. Late-onset Pompe disease (LOPD; including: (a) individuals with onset before age 12 months without cardiomyopathy; and (b) all individuals with onset after age 12 months) is characterized by proximal muscle weakness and respiratory insufficiency; clinically significant cardiac involvement is uncommon.
Myopathy, myosin storage, autosomal recessive
MedGen UID:
340603
Concept ID:
C1850709
Disease or Syndrome
Autosomal recessive myosin storage congenital myopathy-7B (CMYP7B) is a skeletal muscle disorder characterized by the onset of scapuloperoneal muscle weakness in early childhood or young adulthood. Affected individuals have difficulty walking, steppage gait, and scapular winging due to shoulder girdle involvement. The severity and progression of the disorder is highly variable, even within families. Most patients develop respiratory insufficiency, nocturnal hypoventilation, and restrictive lung disease; some develop hypertrophic cardiomyopathy. Additional features include myopathic facies, high-arched palate, scoliosis, and muscle wasting with thin body habitus. Serum creatine kinase may be normal or elevated. Skeletal muscle biopsy shows variable findings, including myosin storage disease, type 1 fiber predominance, centralized nuclei, and multiminicore disease (Onengut et al., 2004; Tajsharghi et al., 2007; Beecroft et al., 2019). For a discussion of genetic heterogeneity of congenital myopathy, see CMYP1A (117000).

Professional guidelines

PubMed

Halasz G, Cattaneo M, Piepoli M, Romano S, Biasini V, Menafoglio A, Gasperetti A, Badini M, Villa M, Dall'Ara L, Roberto M, Cassina T, Capelli B
Int J Cardiol 2021 Jul 15;335:40-46. Epub 2021 Apr 12 doi: 10.1016/j.ijcard.2021.04.019. PMID: 33857542
Nogami A
Card Electrophysiol Rev 2002 Dec;6(4):448-57. doi: 10.1023/a:1021100828459. PMID: 12438827
OLNEY MB
Calif Med 1949 Jun;70(6):453-8. PMID: 18131680Free PMC Article

Recent clinical studies

Etiology

Ackerman LH, Reynolds PS, Aherne M, Swift ST
Am J Vet Res 2022 Jan 12;83(4):312-316. doi: 10.2460/ajvr.21.09.0138. PMID: 35038304
Lui JK, Sangani RA, Chen CA, Bujor AM, Trojanowski MA, Gopal DM, LaValley MP, Soylemez Wiener R, Klings ES
Arthritis Care Res (Hoboken) 2022 Jul;74(7):1219-1226. Epub 2022 Apr 5 doi: 10.1002/acr.24724. PMID: 34085410Free PMC Article
Seko Y, Kato T, Yamaji Y, Haruna Y, Nakane E, Haruna T, Inoko M
Sci Rep 2021 Apr 26;11(1):8892. doi: 10.1038/s41598-021-88259-8. PMID: 33903653Free PMC Article
Stein PD, Matta F, Ekkah M, Saleh T, Janjua M, Patel YR, Khadra H
Am J Cardiol 2012 Dec 15;110(12):1836-40. Epub 2012 Sep 21 doi: 10.1016/j.amjcard.2012.08.019. PMID: 23000104
Glauser J
Cleve Clin J Med 2000 Oct;67(10):704-6, 709-13, 717-9. doi: 10.3949/ccjm.67.10.704. PMID: 11060957

Diagnosis

Ley L, Höltgen R, Bogossian H, Ghofrani HA, Bandorski D
J Electrocardiol 2023 Jul-Aug;79:24-29. Epub 2023 Mar 5 doi: 10.1016/j.jelectrocard.2023.02.007. PMID: 36913785
Lui JK, Sangani RA, Chen CA, Bujor AM, Trojanowski MA, Gopal DM, LaValley MP, Soylemez Wiener R, Klings ES
Arthritis Care Res (Hoboken) 2022 Jul;74(7):1219-1226. Epub 2022 Apr 5 doi: 10.1002/acr.24724. PMID: 34085410Free PMC Article
Bellamoli M, Marin F, Maritan L, Prati D, Tadiello E, Pesarini G, Mugnai G, Ribichini FL, Pighi M
J Electrocardiol 2020 May-Jun;60:60-66. Epub 2020 Mar 9 doi: 10.1016/j.jelectrocard.2020.03.003. PMID: 32272291
Sims JR, Enriquez-Sarano M, Michelena HI
Heart 2017 Mar;103(5):367. Epub 2016 Sep 15 doi: 10.1136/heartjnl-2016-310204. PMID: 27634410
Stein PD, Matta F, Ekkah M, Saleh T, Janjua M, Patel YR, Khadra H
Am J Cardiol 2012 Dec 15;110(12):1836-40. Epub 2012 Sep 21 doi: 10.1016/j.amjcard.2012.08.019. PMID: 23000104

Therapy

Lui JK, Sangani RA, Chen CA, Bujor AM, Trojanowski MA, Gopal DM, LaValley MP, Soylemez Wiener R, Klings ES
Arthritis Care Res (Hoboken) 2022 Jul;74(7):1219-1226. Epub 2022 Apr 5 doi: 10.1002/acr.24724. PMID: 34085410Free PMC Article
Sims JR, Enriquez-Sarano M, Michelena HI
Heart 2017 Mar;103(5):367. Epub 2016 Sep 15 doi: 10.1136/heartjnl-2016-310204. PMID: 27634410
Barold SS, Herweg B
Cardiol J 2011;18(5):476-86. doi: 10.5603/cj.2011.0002. PMID: 21947982
Nogami A
Pacing Clin Electrophysiol 2011 May;34(5):624-50. Epub 2011 Mar 16 doi: 10.1111/j.1540-8159.2011.03044.x. PMID: 21410719
Glauser J
Cleve Clin J Med 2000 Oct;67(10):704-6, 709-13, 717-9. doi: 10.3949/ccjm.67.10.704. PMID: 11060957

Prognosis

Ley L, Höltgen R, Bogossian H, Ghofrani HA, Bandorski D
J Electrocardiol 2023 Jul-Aug;79:24-29. Epub 2023 Mar 5 doi: 10.1016/j.jelectrocard.2023.02.007. PMID: 36913785
Ackerman LH, Reynolds PS, Aherne M, Swift ST
Am J Vet Res 2022 Jan 12;83(4):312-316. doi: 10.2460/ajvr.21.09.0138. PMID: 35038304
Lui JK, Sangani RA, Chen CA, Bujor AM, Trojanowski MA, Gopal DM, LaValley MP, Soylemez Wiener R, Klings ES
Arthritis Care Res (Hoboken) 2022 Jul;74(7):1219-1226. Epub 2022 Apr 5 doi: 10.1002/acr.24724. PMID: 34085410Free PMC Article
Seko Y, Kato T, Yamaji Y, Haruna Y, Nakane E, Haruna T, Inoko M
Sci Rep 2021 Apr 26;11(1):8892. doi: 10.1038/s41598-021-88259-8. PMID: 33903653Free PMC Article
Glauser J
Cleve Clin J Med 2000 Oct;67(10):704-6, 709-13, 717-9. doi: 10.3949/ccjm.67.10.704. PMID: 11060957

Clinical prediction guides

Ley L, Höltgen R, Bogossian H, Ghofrani HA, Bandorski D
J Electrocardiol 2023 Jul-Aug;79:24-29. Epub 2023 Mar 5 doi: 10.1016/j.jelectrocard.2023.02.007. PMID: 36913785
Lui JK, Sangani RA, Chen CA, Bujor AM, Trojanowski MA, Gopal DM, LaValley MP, Soylemez Wiener R, Klings ES
Arthritis Care Res (Hoboken) 2022 Jul;74(7):1219-1226. Epub 2022 Apr 5 doi: 10.1002/acr.24724. PMID: 34085410Free PMC Article
Bellamoli M, Marin F, Maritan L, Prati D, Tadiello E, Pesarini G, Mugnai G, Ribichini FL, Pighi M
J Electrocardiol 2020 May-Jun;60:60-66. Epub 2020 Mar 9 doi: 10.1016/j.jelectrocard.2020.03.003. PMID: 32272291
Stein PD, Matta F, Ekkah M, Saleh T, Janjua M, Patel YR, Khadra H
Am J Cardiol 2012 Dec 15;110(12):1836-40. Epub 2012 Sep 21 doi: 10.1016/j.amjcard.2012.08.019. PMID: 23000104
Glauser J
Cleve Clin J Med 2000 Oct;67(10):704-6, 709-13, 717-9. doi: 10.3949/ccjm.67.10.704. PMID: 11060957

Recent systematic reviews

Minotti B, Brenner R, Desbiolles LM, Osterwalder J, Schoch OD, Ammann P
Swiss Med Wkly 2021 Aug 2;151:w30041. Epub 2021 Aug 3 doi: 10.4414/SMW.2021.w30041. PMID: 34495608
Qaddoura A, Digby GC, Kabali C, Kukla P, Zhan ZQ, Baranchuk AM
Clin Cardiol 2017 Oct;40(10):814-824. Epub 2017 Jun 19 doi: 10.1002/clc.22742. PMID: 28628222Free PMC Article

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