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Right ventricular noncompaction cardiomyopathy

MedGen UID:
868320
Concept ID:
C4022714
Disease or Syndrome
HPO: HP:0012816

Definition

A predominantly right ventricular variant of isolated noncompaction cardiomyopathy. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRight ventricular noncompaction cardiomyopathy

Professional guidelines

PubMed

Wanert C, El Louali F, Al Dybiat S, Nguyen K, Zaffran S, Ovaert C
Arch Cardiovasc Dis 2023 Jun-Jul;116(6-7):309-315. Epub 2023 May 16 doi: 10.1016/j.acvd.2023.04.008. PMID: 37246080
Towbin JA, McKenna WJ, Abrams DJ, Ackerman MJ, Calkins H, Darrieux FCC, Daubert JP, de Chillou C, DePasquale EC, Desai MY, Estes NAM 3rd, Hua W, Indik JH, Ingles J, James CA, John RM, Judge DP, Keegan R, Krahn AD, Link MS, Marcus FI, McLeod CJ, Mestroni L, Priori SG, Saffitz JE, Sanatani S, Shimizu W, van Tintelen JP, Wilde AAM, Zareba W
Heart Rhythm 2019 Nov;16(11):e301-e372. Epub 2019 May 9 doi: 10.1016/j.hrthm.2019.05.007. PMID: 31078652
Kantor PF, Lougheed J, Dancea A, McGillion M, Barbosa N, Chan C, Dillenburg R, Atallah J, Buchholz H, Chant-Gambacort C, Conway J, Gardin L, George K, Greenway S, Human DG, Jeewa A, Price JF, Ross RD, Roche SL, Ryerson L, Soni R, Wilson J, Wong K; Children's Heart Failure Study Group
Can J Cardiol 2013 Dec;29(12):1535-52. doi: 10.1016/j.cjca.2013.08.008. PMID: 24267800

Recent clinical studies

Etiology

Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
Towbin JA, McKenna WJ, Abrams DJ, Ackerman MJ, Calkins H, Darrieux FCC, Daubert JP, de Chillou C, DePasquale EC, Desai MY, Estes NAM 3rd, Hua W, Indik JH, Ingles J, James CA, John RM, Judge DP, Keegan R, Krahn AD, Link MS, Marcus FI, McLeod CJ, Mestroni L, Priori SG, Saffitz JE, Sanatani S, Shimizu W, van Tintelen JP, Wilde AAM, Zareba W
Heart Rhythm 2019 Nov;16(11):e301-e372. Epub 2019 May 9 doi: 10.1016/j.hrthm.2019.05.007. PMID: 31078652
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, Chung WK, Jefferies JL, Rossano JW, Castleberry CD, Addonizio LJ, Lal AK, Lamour JM, Miller EM, Thrush PT, Czachor JD, Razoky H, Hill A, Lipshultz SE
Circ Res 2017 Sep 15;121(7):855-873. doi: 10.1161/CIRCRESAHA.116.309386. PMID: 28912187Free PMC Article
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865

Diagnosis

Towbin JA, McKenna WJ, Abrams DJ, Ackerman MJ, Calkins H, Darrieux FCC, Daubert JP, de Chillou C, DePasquale EC, Desai MY, Estes NAM 3rd, Hua W, Indik JH, Ingles J, James CA, John RM, Judge DP, Keegan R, Krahn AD, Link MS, Marcus FI, McLeod CJ, Mestroni L, Priori SG, Saffitz JE, Sanatani S, Shimizu W, van Tintelen JP, Wilde AAM, Zareba W
Heart Rhythm 2019 Nov;16(11):e301-e372. Epub 2019 May 9 doi: 10.1016/j.hrthm.2019.05.007. PMID: 31078652
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, Chung WK, Jefferies JL, Rossano JW, Castleberry CD, Addonizio LJ, Lal AK, Lamour JM, Miller EM, Thrush PT, Czachor JD, Razoky H, Hill A, Lipshultz SE
Circ Res 2017 Sep 15;121(7):855-873. doi: 10.1161/CIRCRESAHA.116.309386. PMID: 28912187Free PMC Article
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865
Kantor PF, Lougheed J, Dancea A, McGillion M, Barbosa N, Chan C, Dillenburg R, Atallah J, Buchholz H, Chant-Gambacort C, Conway J, Gardin L, George K, Greenway S, Human DG, Jeewa A, Price JF, Ross RD, Roche SL, Ryerson L, Soni R, Wilson J, Wong K; Children's Heart Failure Study Group
Can J Cardiol 2013 Dec;29(12):1535-52. doi: 10.1016/j.cjca.2013.08.008. PMID: 24267800

Therapy

Huo Q, Liang L, Ding X, Pang M, Zhao Y, Zhang H, Su W
Ann Noninvasive Electrocardiol 2020 Sep;25(5):e12731. Epub 2019 Nov 20 doi: 10.1111/anec.12731. PMID: 31746517Free PMC Article
Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, Chung WK, Jefferies JL, Rossano JW, Castleberry CD, Addonizio LJ, Lal AK, Lamour JM, Miller EM, Thrush PT, Czachor JD, Razoky H, Hill A, Lipshultz SE
Circ Res 2017 Sep 15;121(7):855-873. doi: 10.1161/CIRCRESAHA.116.309386. PMID: 28912187Free PMC Article
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865
Kantor PF, Lougheed J, Dancea A, McGillion M, Barbosa N, Chan C, Dillenburg R, Atallah J, Buchholz H, Chant-Gambacort C, Conway J, Gardin L, George K, Greenway S, Human DG, Jeewa A, Price JF, Ross RD, Roche SL, Ryerson L, Soni R, Wilson J, Wong K; Children's Heart Failure Study Group
Can J Cardiol 2013 Dec;29(12):1535-52. doi: 10.1016/j.cjca.2013.08.008. PMID: 24267800
Finsterer J, Stöllberger C, Wahbi K
Cardiovasc Pathol 2013 Sep-Oct;22(5):389-400. Epub 2013 Feb 22 doi: 10.1016/j.carpath.2012.12.008. PMID: 23433859

Prognosis

Wang Y, Jia H, Song J
Curr Cardiol Rep 2023 Oct;25(10):1299-1317. Epub 2023 Sep 15 doi: 10.1007/s11886-023-01944-0. PMID: 37721634Free PMC Article
Fadl SA, Revels JW, Rezai Gharai L, Hanneman K, Dana F, Proffitt EK, Grizzard JD
Radiographics 2022 May-Jun;42(3):625-643. Epub 2022 Mar 11 doi: 10.1148/rg.210147. PMID: 35275782
Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
Lee TM, Hsu DT, Kantor P, Towbin JA, Ware SM, Colan SD, Chung WK, Jefferies JL, Rossano JW, Castleberry CD, Addonizio LJ, Lal AK, Lamour JM, Miller EM, Thrush PT, Czachor JD, Razoky H, Hill A, Lipshultz SE
Circ Res 2017 Sep 15;121(7):855-873. doi: 10.1161/CIRCRESAHA.116.309386. PMID: 28912187Free PMC Article
Kantor PF, Lougheed J, Dancea A, McGillion M, Barbosa N, Chan C, Dillenburg R, Atallah J, Buchholz H, Chant-Gambacort C, Conway J, Gardin L, George K, Greenway S, Human DG, Jeewa A, Price JF, Ross RD, Roche SL, Ryerson L, Soni R, Wilson J, Wong K; Children's Heart Failure Study Group
Can J Cardiol 2013 Dec;29(12):1535-52. doi: 10.1016/j.cjca.2013.08.008. PMID: 24267800

Clinical prediction guides

Stämpfli SF, Gotschy A, Kiarostami P, Özkartal T, Gruner C, Niemann M, Manka R, Tanner FC
Cardiol J 2022;29(3):454-462. Epub 2020 Jul 10 doi: 10.5603/CJ.a2020.0095. PMID: 32648250Free PMC Article
Adabifirouzjaei F, Igata S, DeMaria AN
Prog Cardiovasc Dis 2021 Sep-Oct;68:60-69. Epub 2021 Jul 13 doi: 10.1016/j.pcad.2021.07.007. PMID: 34265334
Stämpfli SF, Donati TG, Hellermann J, Anwer S, Erhart L, Gruner C, Kaufmann BA, Gencer B, Haager PK, Müller H, Tanner FC
J Cardiol 2020 Jan;75(1):20-26. Epub 2019 Oct 4 doi: 10.1016/j.jjcc.2019.09.003. PMID: 31587941
Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
Bang ML
J Cell Physiol 2017 Jan;232(1):38-52. Epub 2016 Jun 2 doi: 10.1002/jcp.25424. PMID: 27171814

Recent systematic reviews

Bazoukis G, Tyrovolas K, Letsas KP, Vlachos K, Radford D, Chung CT, Liu T, Efremidis M, Tse G, Baranchuk A
Heart Fail Rev 2022 Nov;27(6):2067-2076. Epub 2022 Jul 1 doi: 10.1007/s10741-022-10257-3. PMID: 35776368
Bhaskaran A, Campbell T, Virk S, Bennett RG, Kizana E, Kumar S
J Cardiovasc Electrophysiol 2021 May;32(5):1421-1429. Epub 2021 Apr 12 doi: 10.1111/jce.15026. PMID: 33792994
Krul SP, van der Smagt JJ, van den Berg MP, Sollie KM, Pieper PG, van Spaendonck-Zwarts KY
Eur J Heart Fail 2011 Jun;13(6):584-94. Epub 2011 Apr 11 doi: 10.1093/eurjhf/hfr040. PMID: 21482599

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