U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Risk stratification and management of women with cardiomyopathy/heart failure planning pregnancy or presenting during/after pregnancy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy.
Sliwa K, van der Meer P, Petrie MC, Frogoudaki A, Johnson MR, Hilfiker-Kleiner D, Hamdan R, Jackson AM, Ibrahim B, Mbakwem A, Tschöpe C, Regitz-Zagrosek V, Omerovic E, Roos-Hesselink J, Gatzoulis M, Tutarel O, Price S, Heymans S, Coats AJS, Müller C, Chioncel O, Thum T, de Boer RA, Jankowska E, Ponikowski P, Lyon AR, Rosano G, Seferovic PM, Bauersachs J
Eur J Heart Fail 2021 Apr;23(4):527-540. Epub 2021 Mar 17 doi: 10.1002/ejhf.2133. PMID: 33609068
Genome Editing for the Understanding and Treatment of Inherited Cardiomyopathies.
Nguyen Q, Lim KRQ, Yokota T
Int J Mol Sci 2020 Jan 22;21(3) doi: 10.3390/ijms21030733. PMID: 31979133Free 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

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

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
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

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
Left ventricular hypertrabeculation/noncompaction.
Stöllberger C, Finsterer J
J Am Soc Echocardiogr 2004 Jan;17(1):91-100. doi: 10.1016/S0894-7317(03)00514-5. PMID: 14712196

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
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
Right ventricular morphology and systolic function in left ventricular noncompaction cardiomyopathy.
Stacey RB, Andersen M, Haag J, Hall ME, McLeod G, Upadhya B, Hundley WG, Thohan V
Am J Cardiol 2014 Mar 15;113(6):1018-23. Epub 2013 Dec 25 doi: 10.1016/j.amjcard.2013.12.008. PMID: 24462071Free PMC Article

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...