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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

Genetic profile and genotype-phenotype correlations in childhood cardiomyopathy.
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
2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.
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
Inherited cardiac arrhythmias: diagnosis, treatment, and prevention.
Beckmann BM, Pfeufer A, Kääb S
Dtsch Arztebl Int 2011 Sep;108(37):623-33; quiz 634. Epub 2011 Sep 16 doi: 10.3238/arztebl.2011.0623. PMID: 21977220Free PMC Article

Recent clinical studies

Etiology

Cardiomyopathy and pregnancy.
Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.
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
Cardiomyopathy: An Overview.
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
Pediatric Cardiomyopathies.
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
Left ventricular non-compaction cardiomyopathy.
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

Cardiomyopathies in children: An overview.
Tsatsopoulou A, Protonotarios I, Xylouri Z, Papagiannis I, Anastasakis A, Germanakis I, Patrianakos A, Nyktari E, Gavras C, Papadopoulos G, Meditskou S, Lazarou E, Miliou A, Lazaros G
Hellenic J Cardiol 2023 Jul-Aug;72:43-56. Epub 2023 Mar 3 doi: 10.1016/j.hjc.2023.02.007. PMID: 36870438
2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.
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
Cardiomyopathy: An Overview.
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
Pediatric Cardiomyopathies.
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
Left ventricular non-compaction cardiomyopathy.
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

Therapy

Isolated right ventricular noncompaction caused ventricular tachycardia and pulmonary embolism.
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
Pediatric Cardiomyopathies.
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
Left ventricular non-compaction cardiomyopathy.
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
Isolated left ventricular noncompaction causing stroke in a 30-year-old woman: case report and literature review.
Baquero GA, Colegrove DJ, Banchs JE
Tex Heart Inst J 2013;40(3):331-8. PMID: 23914034Free PMC Article
Cardiomyopathy in neurological disorders.
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

Accurate Classification of Non-ischemic Cardiomyopathy.
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
Cardiac MRI of Hereditary Cardiomyopathy.
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
Right ventricular involvement in left ventricular non-compaction cardiomyopathy.
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
Cardiomyopathy and pregnancy.
Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
Pediatric Cardiomyopathies.
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

Clinical prediction guides

Right ventricular involvement in left ventricular non-compaction cardiomyopathy.
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
Hypertrabeculation; a phenotype with Heterogeneous etiology.
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
Right ventricle and outcome in left ventricular non-compaction cardiomyopathy.
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
Cardiomyopathy and pregnancy.
Schaufelberger M
Heart 2019 Oct;105(20):1543-1551. Epub 2019 Jul 15 doi: 10.1136/heartjnl-2018-313476. PMID: 31308064Free PMC Article
Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins.
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

Predictors of fatal arrhythmic events in patients with non-compaction cardiomyopathy: a systematic review.
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
Electrophysiologic and electroanatomic characterization of ventricular arrhythmias in non-compaction cardiomyopathy: A systematic review.
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
Systematic review of pregnancy in women with inherited cardiomyopathies.
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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