U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Isolated Noncompaction of the Ventricular Myocardium

MedGen UID:
403467
•Concept ID:
C2717907
•
Disease or Syndrome
Synonym: Isolated Non-compaction of the Ventricular Myocardium

Definition

Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. Often, the RIGHT VENTRICLE is also affected. CONGESTIVE HEART FAILURE; PULMONARY EMBOLISM; and ventricular ARRHYTHMIA are commonly seen. [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Isolated Noncompaction of the Ventricular Myocardium

Professional guidelines

PubMed

Left Ventricular Noncompaction Detected by Cardiac Magnetic Resonance Screening: A Reexamination of Diagnostic Criteria.
Masso AH, Uribe C, Willerson JT, Cheong BY, Davis BR
Tex Heart Inst J 2020 Jun 1;47(3):183-193. doi: 10.14503/THIJ-19-7157. PMID: 32997774Free PMC Article

Recent clinical studies

Etiology

Ventricular tachycardia due to isolated non-compaction of the right ventricle.
Rambhatla T, Mountantonakis S, Bhasin K, Skipitaris N, Kronzon I
Eur Heart J Cardiovasc Imaging 2018 Aug 1;19(8):878. doi: 10.1093/ehjci/jey068. PMID: 29757364
Genetics, Clinical Features, and Long-Term Outcome of Noncompaction Cardiomyopathy.
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
Isolated Noncompaction of the Left Ventricle in Adults.
Hussein A, Karimianpour A, Collier P, Krasuski RA
J Am Coll Cardiol 2015 Aug 4;66(5):578-85. doi: 10.1016/j.jacc.2015.06.017. PMID: 26227197
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
Cardiomyopathy in childhood.
Williams GD, Hammer GB
Curr Opin Anaesthesiol 2011 Jun;24(3):289-300. doi: 10.1097/ACO.0b013e3283462257. PMID: 21478741

Diagnosis

Excessive Trabeculation of the Left Ventricle: JACC: Cardiovascular Imaging Expert Panel Paper.
Petersen SE, Jensen B, Aung N, Friedrich MG, McMahon CJ, Mohiddin SA, Pignatelli RH, Ricci F, Anderson RH, Bluemke DA
JACC Cardiovasc Imaging 2023 Mar;16(3):408-425. Epub 2023 Feb 8 doi: 10.1016/j.jcmg.2022.12.026. PMID: 36764891Free PMC Article
Ventricular non-compaction review.
Srivastava S, Yavari M, Al-Abcha A, Banga S, Abela G
Heart Fail Rev 2022 Jul;27(4):1063-1076. Epub 2021 Jul 7 doi: 10.1007/s10741-021-10128-3. PMID: 34232438
Genetics, Clinical Features, and Long-Term Outcome of Noncompaction Cardiomyopathy.
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
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
Foot-like heart.
Andreou I, Tentolouris C, Brili S, Tousoulis D, Stefanadis C
Lancet 2013 May 25;381(9880):1844. Epub 2012 Dec 14 doi: 10.1016/S0140-6736(12)61337-3. PMID: 23245994

Therapy

Anticoagulation Therapy in Specific Cardiomyopathies: Isolated Left Ventricular Noncompaction and Peripartum Cardiomyopathy.
Kido K, Guglin M
J Cardiovasc Pharmacol Ther 2019 Jan;24(1):31-36. Epub 2018 Jun 18 doi: 10.1177/1074248418783745. PMID: 29911432
Masking and Unmasking of Isolated Noncompaction of the Left Ventricle With Real-Time Contrast Echocardiography.
Aggarwal S, Xie F, Porter TR
Circ Cardiovasc Imaging 2017 Nov;10(11) doi: 10.1161/CIRCIMAGING.117.006999. PMID: 29133480
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
Left ventricular non-compaction and dyssynchrony.
Fujikura K, Finkielstein D, Rachko M, Garcia MJ
Eur Heart J Cardiovasc Imaging 2013 Nov;14(11):1117. Epub 2013 May 21 doi: 10.1093/ehjci/jet098. PMID: 23696559
The treatment of noncompaction of ventricular myocardium.
Song ZZ
Cardiovasc Ther 2011 Oct;29(5):315-21. Epub 2010 Jul 7 doi: 10.1111/j.1755-5922.2010.00174.x. PMID: 20626405

Prognosis

Excessive Trabeculation of the Left Ventricle: JACC: Cardiovascular Imaging Expert Panel Paper.
Petersen SE, Jensen B, Aung N, Friedrich MG, McMahon CJ, Mohiddin SA, Pignatelli RH, Ricci F, Anderson RH, Bluemke DA
JACC Cardiovasc Imaging 2023 Mar;16(3):408-425. Epub 2023 Feb 8 doi: 10.1016/j.jcmg.2022.12.026. PMID: 36764891Free PMC Article
Ventricular tachycardia due to isolated non-compaction of the right ventricle.
Rambhatla T, Mountantonakis S, Bhasin K, Skipitaris N, Kronzon I
Eur Heart J Cardiovasc Imaging 2018 Aug 1;19(8):878. doi: 10.1093/ehjci/jey068. PMID: 29757364
Genetics, Clinical Features, and Long-Term Outcome of Noncompaction Cardiomyopathy.
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
A noncompaction reaction.
Alazzoni A, Nair V, Valettas N, Salehian O
Circulation 2014 May 27;129(21):2171-8. doi: 10.1161/CIRCULATIONAHA.114.009693. PMID: 24867996
Cardiomyopathy in childhood.
Williams GD, Hammer GB
Curr Opin Anaesthesiol 2011 Jun;24(3):289-300. doi: 10.1097/ACO.0b013e3283462257. PMID: 21478741

Clinical prediction guides

Excessive Trabeculation of the Left Ventricle: JACC: Cardiovascular Imaging Expert Panel Paper.
Petersen SE, Jensen B, Aung N, Friedrich MG, McMahon CJ, Mohiddin SA, Pignatelli RH, Ricci F, Anderson RH, Bluemke DA
JACC Cardiovasc Imaging 2023 Mar;16(3):408-425. Epub 2023 Feb 8 doi: 10.1016/j.jcmg.2022.12.026. PMID: 36764891Free PMC Article
Ventricular non-compaction review.
Srivastava S, Yavari M, Al-Abcha A, Banga S, Abela G
Heart Fail Rev 2022 Jul;27(4):1063-1076. Epub 2021 Jul 7 doi: 10.1007/s10741-021-10128-3. PMID: 34232438
Genetics, Clinical Features, and Long-Term Outcome of Noncompaction Cardiomyopathy.
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
Masking and Unmasking of Isolated Noncompaction of the Left Ventricle With Real-Time Contrast Echocardiography.
Aggarwal S, Xie F, Porter TR
Circ Cardiovasc Imaging 2017 Nov;10(11) doi: 10.1161/CIRCIMAGING.117.006999. PMID: 29133480
Tiger heart: a variant of isolated left ventricular noncompaction?
Parameswaran AC, Cheong BY
Tex Heart Inst J 2012;39(3):444-5. PMID: 22719167Free PMC Article

Recent systematic reviews

Electrophysiological phenotyping of left ventricular noncompaction cardiomyopathy in pediatric populations: A systematic review.
Fitzsimons LA, Kneeland-Barber DM, Hannigan GC, Karpe DA, Wu L, Colon M, Randall J, Tucker KL
Physiol Rep 2024 May;12(9):e16029. doi: 10.14814/phy2.16029. PMID: 38684446Free PMC Article
Prognostic Significance of Left Ventricular Noncompaction: Systematic Review and Meta-Analysis of Observational Studies.
Aung N, Doimo S, Ricci F, Sanghvi MM, Pedrosa C, Woodbridge SP, Al-Balah A, Zemrak F, Khanji MY, Munroe PB, Naci H, Petersen SE
Circ Cardiovasc Imaging 2020 Jan;13(1):e009712. Epub 2020 Jan 21 doi: 10.1161/CIRCIMAGING.119.009712. PMID: 31959004Free PMC Article
A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults.
Ross SB, Jones K, Blanch B, Puranik R, McGeechan K, Barratt A, Semsarian C
Eur Heart J 2020 Apr 7;41(14):1428-1436. doi: 10.1093/eurheartj/ehz317. PMID: 31143950
Clinical and genetic insights into non-compaction: a meta-analysis and systematic review on 7598 individuals.
Kayvanpour E, Sedaghat-Hamedani F, Gi WT, Tugrul OF, Amr A, Haas J, Zhu F, Ehlermann P, Uhlmann L, Katus HA, Meder B
Clin Res Cardiol 2019 Nov;108(11):1297-1308. Epub 2019 Apr 12 doi: 10.1007/s00392-019-01465-3. PMID: 30980206
Anticoagulation Therapy in Specific Cardiomyopathies: Isolated Left Ventricular Noncompaction and Peripartum Cardiomyopathy.
Kido K, Guglin M
J Cardiovasc Pharmacol Ther 2019 Jan;24(1):31-36. Epub 2018 Jun 18 doi: 10.1177/1074248418783745. PMID: 29911432

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

    • ClinVar
      Related medical variations
    • MeSH
      Related Medical Subject Headings
    • 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...