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Non-compaction cardiomyopathy

MedGen UID:
1843481
•Concept ID:
C4324548
•
Disease or Syndrome
Synonym: non-compaction cardiomyopathy
 
Monarch Initiative: MONDO:0005418

Definition

Left ventricular non-compaction (LVNC) is characterized by prominent left ventricular trabeculae and deep inter-trabecular recesses. The myocardial wall is often thickened with a thin, compacted epicardial layer and a thickened endocardial layer. In some patients, LVNC is associated with left ventricular dilatation and systolic dysfunction, which can be transient in neonates. [from MONDO]

Professional guidelines

PubMed

Genetic Testing as a Guide for Treatment in Dilated Cardiomyopathies.
García-Hernandez S, Iglesias LM
Curr Cardiol Rep 2022 Nov;24(11):1537-1546. Epub 2022 Aug 22 doi: 10.1007/s11886-022-01772-8. PMID: 35994197
Hypertrophic cardiomyopathy, non-compaction cardiomyopathy or non-compaction phenotype - another diagnosis, other further treatment.
Bednarek A, Stec M, Mizia-Szubryt M, Cichoń M, Kuczmik W, Mizia-Stec K
Kardiol Pol 2021;79(6):700-701. Epub 2021 May 20 doi: 10.33963/KP.15935. PMID: 33871231
Comment on "Ventricular non-compaction cardiomyopathy: prenatal diagnosis and pathology".
Stöllberger C, Finsterer J
Prenat Diagn 2016 Feb;36(2):197-8. doi: 10.1002/pd.4699. PMID: 26865063

Recent clinical studies

Etiology

Advanced Heart Failure in Special Population: Cardiomyopathies and Myocarditis.
Stolfo D, Collini V, Sinagra G
Heart Fail Clin 2021 Oct;17(4):661-672. doi: 10.1016/j.hfc.2021.05.010. PMID: 34511213
Ventricular fibrillation undersensing in a patient with left ventricular non-compaction cardiomyopathy and a cardiac resynchronization therapy implantable defibrillator (CRT-D).
Capulzini L, Sorgente A
J Interv Card Electrophysiol 2021 Apr;60(3):559-560. Epub 2020 Sep 23 doi: 10.1007/s10840-020-00883-y. PMID: 32968864
Dilated cardiomyopathies and non-compaction cardiomyopathy.
Hänselmann A, Veltmann C, Bauersachs J, Berliner D
Herz 2020 May;45(3):212-220. doi: 10.1007/s00059-020-04903-5. PMID: 32107565Free 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
Non-compaction cardiomyopathy.
Almeida AG, Pinto FJ
Heart 2013 Oct;99(20):1535-42. Epub 2013 Mar 16 doi: 10.1136/heartjnl-2012-302048. PMID: 23503402

Diagnosis

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 non-compaction cardiomyopathy: how many needles in the haystack?
D'Silva A, Jensen B
Heart 2021 Aug;107(16):1344-1352. Epub 2020 Nov 5 doi: 10.1136/heartjnl-2020-316945. PMID: 33153995
Non-compaction cardiomyopathy.
Lim A, Langlois N
Forensic Sci Med Pathol 2016 Mar;12(1):104-7. Epub 2016 Jan 23 doi: 10.1007/s12024-016-9743-7. PMID: 26803832
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
Non-compaction cardiomyopathy.
Almeida AG, Pinto FJ
Heart 2013 Oct;99(20):1535-42. Epub 2013 Mar 16 doi: 10.1136/heartjnl-2012-302048. PMID: 23503402

Therapy

Enterocutaneous fistula formation after cardiac transplantation due to injury from LVAD driveline migration.
Tan DW, Yan CL, Yeh DD, Thakkar-Rivera N
BMJ Case Rep 2023 Jun 22;16(6) doi: 10.1136/bcr-2023-254696. PMID: 37348927Free PMC Article
CMR findings after COVID-19 and after COVID-19-vaccination-same but different?
Doeblin P, Jahnke C, Schneider M, Al-Tabatabaee S, Goetze C, Weiss KJ, Tanacli R, Faragli A, Witt U, Stehning C, Seidel F, Elsanhoury A, Kühne T, Tschöpe C, Pieske B, Kelle S
Int J Cardiovasc Imaging 2022 Sep;38(9):2057-2071. Epub 2022 May 12 doi: 10.1007/s10554-022-02623-x. PMID: 37726611Free PMC Article
Non compaction cardiomyopathy: Review of a controversial entity.
Lorca R, Rozado J, Martín M
Med Clin (Barc) 2018 May 11;150(9):354-360. Epub 2017 Nov 22 doi: 10.1016/j.medcli.2017.09.026. PMID: 29173988
Regression of left ventricular hypertrabeculation is associated with improvement in systolic function and favorable prognosis in adult patients with non-ischemic cardiomyopathy.
Minamisawa M, Koyama J, Kozuka A, Miura T, Ebisawa S, Motoki H, Okada A, Izawa A, Ikeda U
J Cardiol 2016 Nov;68(5):431-438. Epub 2015 Dec 28 doi: 10.1016/j.jjcc.2015.11.008. PMID: 26740463
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

Prognosis

Successful pregnancy course and outcome in a patient with unusual coincidence of two structural heart defects: Ebstein anomaly and biventricular non-compaction cardiomyopathy - extremely rare, but there!
Kudlicki J, Styczeń A, Zawiślak J, Artykiewicz-Zawiślak K, Tomaszewski A, Czekajska-Chehab E, Zapolski T
Kardiol Pol 2023;81(12):1286-1287. doi: 10.33963/v.kp.98283. PMID: 38189509
Advanced Heart Failure in Special Population: Cardiomyopathies and Myocarditis.
Stolfo D, Collini V, Sinagra G
Heart Fail Clin 2021 Oct;17(4):661-672. doi: 10.1016/j.hfc.2021.05.010. PMID: 34511213
3D whole heart imaging in severe funnel chest and non-compaction cardiomyopathy.
Polacin M, Biaggi P, Alkadhi H, Kozerke S, Manka R
Int J Cardiovasc Imaging 2021 Feb;37(2):633-634. Epub 2020 Sep 22 doi: 10.1007/s10554-020-02030-0. PMID: 32964328Free PMC Article
Dilated cardiomyopathies and non-compaction cardiomyopathy.
Hänselmann A, Veltmann C, Bauersachs J, Berliner D
Herz 2020 May;45(3):212-220. doi: 10.1007/s00059-020-04903-5. PMID: 32107565Free PMC Article
Isolated non-compaction cardiomyopathy.
Engberding R, Stöllberger C, Ong P, Yelbuz TM, Gerecke BJ, Breithardt G
Dtsch Arztebl Int 2010 Mar;107(12):206-13. Epub 2010 Mar 26 doi: 10.3238/arztebl.2010.0206. PMID: 20386670Free PMC Article

Clinical prediction guides

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
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
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
3D whole heart imaging in severe funnel chest and non-compaction cardiomyopathy.
Polacin M, Biaggi P, Alkadhi H, Kozerke S, Manka R
Int J Cardiovasc Imaging 2021 Feb;37(2):633-634. Epub 2020 Sep 22 doi: 10.1007/s10554-020-02030-0. PMID: 32964328Free PMC Article
Intellectual disability and non-compaction cardiomyopathy with a de novo NONO mutation identified by exome sequencing.
Reinstein E, Tzur S, Cohen R, Bormans C, Behar DM
Eur J Hum Genet 2016 Nov;24(11):1635-1638. Epub 2016 Jun 22 doi: 10.1038/ejhg.2016.72. PMID: 27329731Free PMC Article

Recent systematic reviews

A novel AGK splicing mutation in a patient with Sengers syndrome and left ventricular non-compaction cardiomyopathy.
Fan P, Yang KQ, Han B, Kong D, Yin WH, Li JH, Yang ZX, Niu LL, Fu CS, Rong CZ, Lin YH, Wang H, Zhou XL, Gao LG, Qin XC, Tian T
Pediatr Res 2023 Aug;94(2):683-690. Epub 2023 Feb 9 doi: 10.1038/s41390-023-02515-3. PMID: 36759750
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
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
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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