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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  
  • CROGVIsolated 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

Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.
Casas G, Limeres J, Oristrell G, Gutierrez-Garcia L, Andreini D, Borregan M, Larrañaga-Moreira JM, Lopez-Sainz A, Codina-Solà M, Teixido-Tura G, Sorolla-Romero JA, Fernández-Álvarez P, González-Carrillo J, Guala A, La Mura L, Soler-Fernández R, Sao Avilés A, Santos-Mateo JJ, Marsal JR, Ribera A, de la Pompa JL, Villacorta E, Jiménez-Jáimez J, Ripoll-Vera T, Bayes-Genis A, Garcia-Pinilla JM, Palomino-Doza J, Tiron C, Pontone G, Bogaert J, Aquaro GD, Gimeno-Blanes JR, Zorio E, Garcia-Pavia P, Barriales-Villa R, Evangelista A, Masci PG, Ferreira-González I, Rodríguez-Palomares JF
J Am Coll Cardiol 2021 Aug 17;78(7):643-662. doi: 10.1016/j.jacc.2021.06.016. PMID: 34384546
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
Cardiomyopathy in childhood.
Williams GD, Hammer GB
Curr Opin Anaesthesiol 2011 Jun;24(3):289-300. doi: 10.1097/ACO.0b013e3283462257. PMID: 21478741
Left ventricular noncompaction.
Sarma RJ, Chana A, Elkayam U
Prog Cardiovasc Dis 2010 Jan-Feb;52(4):264-73. doi: 10.1016/j.pcad.2009.11.001. PMID: 20109597

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
Left Ventricular Noncompaction: New Insights into a Poorly Understood Disease.
Filho DCS, do Rêgo Aquino PL, de Souza Silva G, Fabro CB
Curr Cardiol Rev 2021;17(2):209-216. doi: 10.2174/1573403X16666200716151015. PMID: 32674738Free PMC Article
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

Therapy

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 noncompaction of the left ventricular myocardium in adults: a systematic overview.
Bhatia NL, Tajik AJ, Wilansky S, Steidley DE, Mookadam F
J Card Fail 2011 Sep;17(9):771-8. doi: 10.1016/j.cardfail.2011.05.002. PMID: 21872148
Left ventricular noncompaction.
Mangrolia N, Watson T, Gopalan D, Ray KK
J Cardiovasc Med (Hagerstown) 2011 Jun;12(6):430-3. doi: 10.2459/JCM.0b013e3283410295. PMID: 21119528
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
Isolated noncompaction of the ventricular myocardium: contemporary diagnosis and management.
Chrissoheris MP, Ali R, Vivas Y, Marieb M, Protopapas Z
Clin Cardiol 2007 Apr;30(4):156-60. doi: 10.1002/clc.20004. PMID: 17443648Free PMC Article

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
Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.
Casas G, Limeres J, Oristrell G, Gutierrez-Garcia L, Andreini D, Borregan M, Larrañaga-Moreira JM, Lopez-Sainz A, Codina-Solà M, Teixido-Tura G, Sorolla-Romero JA, Fernández-Álvarez P, González-Carrillo J, Guala A, La Mura L, Soler-Fernández R, Sao Avilés A, Santos-Mateo JJ, Marsal JR, Ribera A, de la Pompa JL, Villacorta E, Jiménez-Jáimez J, Ripoll-Vera T, Bayes-Genis A, Garcia-Pinilla JM, Palomino-Doza J, Tiron C, Pontone G, Bogaert J, Aquaro GD, Gimeno-Blanes JR, Zorio E, Garcia-Pavia P, Barriales-Villa R, Evangelista A, Masci PG, Ferreira-González I, Rodríguez-Palomares JF
J Am Coll Cardiol 2021 Aug 17;78(7):643-662. doi: 10.1016/j.jacc.2021.06.016. PMID: 34384546
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
Cardiomyopathy in childhood.
Williams GD, Hammer GB
Curr Opin Anaesthesiol 2011 Jun;24(3):289-300. doi: 10.1097/ACO.0b013e3283462257. PMID: 21478741
Left ventricular noncompaction.
Sarma RJ, Chana A, Elkayam U
Prog Cardiovasc Dis 2010 Jan-Feb;52(4):264-73. doi: 10.1016/j.pcad.2009.11.001. PMID: 20109597

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
Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.
Casas G, Limeres J, Oristrell G, Gutierrez-Garcia L, Andreini D, Borregan M, Larrañaga-Moreira JM, Lopez-Sainz A, Codina-Solà M, Teixido-Tura G, Sorolla-Romero JA, Fernández-Álvarez P, González-Carrillo J, Guala A, La Mura L, Soler-Fernández R, Sao Avilés A, Santos-Mateo JJ, Marsal JR, Ribera A, de la Pompa JL, Villacorta E, Jiménez-Jáimez J, Ripoll-Vera T, Bayes-Genis A, Garcia-Pinilla JM, Palomino-Doza J, Tiron C, Pontone G, Bogaert J, Aquaro GD, Gimeno-Blanes JR, Zorio E, Garcia-Pavia P, Barriales-Villa R, Evangelista A, Masci PG, Ferreira-González I, Rodríguez-Palomares JF
J Am Coll Cardiol 2021 Aug 17;78(7):643-662. doi: 10.1016/j.jacc.2021.06.016. PMID: 34384546
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 noncompaction.
Sarma RJ, Chana A, Elkayam U
Prog Cardiovasc Dis 2010 Jan-Feb;52(4):264-73. doi: 10.1016/j.pcad.2009.11.001. PMID: 20109597

Recent systematic reviews

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
Sports participation in non-compaction cardiomyopathy: a systematic review.
Ganga HV, Thompson PD
Br J Sports Med 2014 Oct;48(20):1466-71. Epub 2013 Mar 19 doi: 10.1136/bjsports-2012-091855. PMID: 23511696

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