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Focal necrosis of right ventricular muscle cells

MedGen UID:
871154
Concept ID:
C4025627
Pathologic Function
HPO: HP:0003338

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal necrosis of right ventricular muscle cells

Conditions with this feature

Arrhythmogenic right ventricular dysplasia 4
MedGen UID:
356107
Concept ID:
C1865881
Disease or Syndrome
Arrhythmogenic right ventricular cardiomyopathy (ARVC) – previously referred to as arrhythmogenic right ventricular dysplasia (ARVD) – is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. It primarily affects the right ventricle, and it may also involve the left ventricle. The presentation of disease is highly variable even within families, and some affected individuals may not meet established clinical criteria. The mean age at diagnosis is 31 years (±13; range: 4-64 years).
Arrhythmogenic right ventricular dysplasia 3
MedGen UID:
356108
Concept ID:
C1865882
Disease or Syndrome
Arrhythmogenic right ventricular cardiomyopathy (ARVC) – previously referred to as arrhythmogenic right ventricular dysplasia (ARVD) – is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. It primarily affects the right ventricle, and it may also involve the left ventricle. The presentation of disease is highly variable even within families, and some affected individuals may not meet established clinical criteria. The mean age at diagnosis is 31 years (±13; range: 4-64 years).

Recent clinical studies

Diagnosis

Okamoto R, Goto I, Nishimura Y, Kobayashi I, Hashizume R, Yoshida Y, Ito R, Kobayashi Y, Nishikawa M, Ali Y, Saito S, Tanaka T, Sawa Y, Ito M, Dohi K
PLoS One 2020;15(10):e0240129. Epub 2020 Oct 13 doi: 10.1371/journal.pone.0240129. PMID: 33048975Free PMC Article
Tang L, Deng C, Long M, Tang A, Wu S, Dong Y, Saravolatz LD, Gardin JM
Mol Med 2008 Mar-Apr;14(3-4):131-40. doi: 10.2119/2007-00097.Tang. PMID: 18224254Free PMC Article

Therapy

Sheppard MN, Cane P, Florio R, Kavantzas N, Close L, Shah J, Lee P, Elliott P
Cardiovasc Pathol 2010 Sep-Oct;19(5):293-301. Epub 2009 Jul 23 doi: 10.1016/j.carpath.2009.05.003. PMID: 19631563

Prognosis

Borchard F
Basic Res Cardiol 1980 Jan-Feb;75(1):118-25. doi: 10.1007/BF02001403. PMID: 6104485

Clinical prediction guides

Okamoto R, Goto I, Nishimura Y, Kobayashi I, Hashizume R, Yoshida Y, Ito R, Kobayashi Y, Nishikawa M, Ali Y, Saito S, Tanaka T, Sawa Y, Ito M, Dohi K
PLoS One 2020;15(10):e0240129. Epub 2020 Oct 13 doi: 10.1371/journal.pone.0240129. PMID: 33048975Free PMC Article
Kant S, Holthöfer B, Magin TM, Krusche CA, Leube RE
Circ Cardiovasc Genet 2015 Aug;8(4):553-63. Epub 2015 Jun 17 doi: 10.1161/CIRCGENETICS.114.000974. PMID: 26085008
Tang L, Deng C, Long M, Tang A, Wu S, Dong Y, Saravolatz LD, Gardin JM
Mol Med 2008 Mar-Apr;14(3-4):131-40. doi: 10.2119/2007-00097.Tang. PMID: 18224254Free PMC Article

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