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Fibrofatty replacement of right ventricular myocardium

MedGen UID:
369873
Concept ID:
C1968863
Pathologic Function
HPO: HP:0034364

Definition

Replacement of the myocardium of the right ventricular free wall by fat and fibrous tissue. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrofatty replacement of right ventricular myocardium

Conditions with this feature

Arrhythmogenic right ventricular dysplasia 9
MedGen UID:
373205
Concept ID:
C1836906
Disease or Syndrome
ARVC may not cause any symptoms in its early stages. However, affected individuals may still be at risk of sudden death, especially during strenuous exercise. When symptoms occur, they most commonly include a sensation of fluttering or pounding in the chest (palpitations), light-headedness, and fainting (syncope). Over time, ARVC can also cause shortness of breath and abnormal swelling in the legs or abdomen. If the myocardium becomes severely damaged in the later stages of the disease, it can lead to heart failure.\n\nArrhythmogenic right ventricular cardiomyopathy (ARVC) is a form of heart disease that usually appears in adulthood. ARVC is a disorder of the myocardium, which is the muscular wall of the heart. This condition causes part of the myocardium to break down over time, increasing the risk of an abnormal heartbeat (arrhythmia) and sudden death.

Professional guidelines

PubMed

Silvano M, Mastella G, Zorzi A, Migliore F, Pilichou K, Bauce B, Rigato I, Perazzolo Marra M, Iliceto S, Thiene G, Basso C, Corrado D
Minerva Med 2016 Aug;107(4):194-216. Epub 2016 May 17 PMID: 27186923

Recent clinical studies

Etiology

Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD
Heart 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612. PMID: 37433658
Trancuccio A, Kukavica D, Sugamiele A, Mazzanti A, Priori SG
Card Electrophysiol Clin 2023 Sep;15(3):349-365. Epub 2023 Jun 9 doi: 10.1016/j.ccep.2023.04.004. PMID: 37558305
Welkie R
JAAPA 2023 May 1;36(5):1-6. doi: 10.1097/01.JAA.0000918764.35264.75. PMID: 37097786
Corrado D, Basso C, Judge DP
Circ Res 2017 Sep 15;121(7):784-802. doi: 10.1161/CIRCRESAHA.117.309345. PMID: 28912183
Pilichou K, Thiene G, Bauce B, Rigato I, Lazzarini E, Migliore F, Perazzolo Marra M, Rizzo S, Zorzi A, Daliento L, Corrado D, Basso C
Orphanet J Rare Dis 2016 Apr 2;11:33. doi: 10.1186/s13023-016-0407-1. PMID: 27038780Free PMC Article

Diagnosis

Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD
Heart 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612. PMID: 37433658
Cunningham SM, Dos Santos L
J Vet Cardiol 2022 Apr;40:156-169. Epub 2021 Jul 23 doi: 10.1016/j.jvc.2021.07.001. PMID: 34503916
Corrado D, Perazzolo Marra M, Zorzi A, Beffagna G, Cipriani A, Lazzari M, Migliore F, Pilichou K, Rampazzo A, Rigato I, Rizzo S, Thiene G, Anastasakis A, Asimaki A, Bucciarelli-Ducci C, Haugaa KH, Marchlinski FE, Mazzanti A, McKenna WJ, Pantazis A, Pelliccia A, Schmied C, Sharma S, Wichter T, Bauce B, Basso C
Int J Cardiol 2020 Nov 15;319:106-114. Epub 2020 Jun 16 doi: 10.1016/j.ijcard.2020.06.005. PMID: 32561223
Corrado D, Basso C, Judge DP
Circ Res 2017 Sep 15;121(7):784-802. doi: 10.1161/CIRCRESAHA.117.309345. PMID: 28912183
Pilichou K, Thiene G, Bauce B, Rigato I, Lazzarini E, Migliore F, Perazzolo Marra M, Rizzo S, Zorzi A, Daliento L, Corrado D, Basso C
Orphanet J Rare Dis 2016 Apr 2;11:33. doi: 10.1186/s13023-016-0407-1. PMID: 27038780Free PMC Article

Therapy

Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD
Heart 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612. PMID: 37433658
Trancuccio A, Kukavica D, Sugamiele A, Mazzanti A, Priori SG
Card Electrophysiol Clin 2023 Sep;15(3):349-365. Epub 2023 Jun 9 doi: 10.1016/j.ccep.2023.04.004. PMID: 37558305
Corrado D, Basso C, Judge DP
Circ Res 2017 Sep 15;121(7):784-802. doi: 10.1161/CIRCRESAHA.117.309345. PMID: 28912183
Padrón-Barthe L, Domínguez F, Garcia-Pavia P, Lara-Pezzi E
Basic Res Cardiol 2017 Sep;112(5):50. Epub 2017 Jul 7 doi: 10.1007/s00395-017-0640-3. PMID: 28688053
Basso C, Corrado D, Marcus FI, Nava A, Thiene G
Lancet 2009 Apr 11;373(9671):1289-300. doi: 10.1016/S0140-6736(09)60256-7. PMID: 19362677

Prognosis

Welkie R
JAAPA 2023 May 1;36(5):1-6. doi: 10.1097/01.JAA.0000918764.35264.75. PMID: 37097786
Cunningham SM, Dos Santos L
J Vet Cardiol 2022 Apr;40:156-169. Epub 2021 Jul 23 doi: 10.1016/j.jvc.2021.07.001. PMID: 34503916
James CA, Calkins H
Annu Rev Med 2019 Jan 27;70:1-18. Epub 2018 Oct 24 doi: 10.1146/annurev-med-041217-010932. PMID: 30355260
Corrado D, Basso C, Judge DP
Circ Res 2017 Sep 15;121(7):784-802. doi: 10.1161/CIRCRESAHA.117.309345. PMID: 28912183
Que D, Yang P, Song X, Liu L
Europace 2015 Dec;17(12):1770-6. Epub 2015 Apr 28 doi: 10.1093/europace/euv042. PMID: 25921558

Clinical prediction guides

James CA, Calkins H
Annu Rev Med 2019 Jan 27;70:1-18. Epub 2018 Oct 24 doi: 10.1146/annurev-med-041217-010932. PMID: 30355260
Corrado D, Basso C, Judge DP
Circ Res 2017 Sep 15;121(7):784-802. doi: 10.1161/CIRCRESAHA.117.309345. PMID: 28912183
Silvano M, Mastella G, Zorzi A, Migliore F, Pilichou K, Bauce B, Rigato I, Perazzolo Marra M, Iliceto S, Thiene G, Basso C, Corrado D
Minerva Med 2016 Aug;107(4):194-216. Epub 2016 May 17 PMID: 27186923
Te Riele ASJM, Tandri H, Sanborn DM, Bluemke DA
JACC Cardiovasc Imaging 2015 May;8(5):597-611. doi: 10.1016/j.jcmg.2015.02.007. PMID: 25937197Free PMC Article
Que D, Yang P, Song X, Liu L
Europace 2015 Dec;17(12):1770-6. Epub 2015 Apr 28 doi: 10.1093/europace/euv042. PMID: 25921558

Recent systematic reviews

Cianci V, Forzese E, Sapienza D, Cianci A, Ieni A, Germanà A, Guerrera MC, Omero F, Speranza D, Cracò A, Asmundo A, Gualniera P, Mondello C
Int J Mol Sci 2024 Feb 20;25(5) doi: 10.3390/ijms25052467. PMID: 38473714Free PMC Article
James CA, Calkins H
Annu Rev Med 2019 Jan 27;70:1-18. Epub 2018 Oct 24 doi: 10.1146/annurev-med-041217-010932. PMID: 30355260

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