U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Left ventricular noncompaction 9(LVNC9)

MedGen UID:
814475
•Concept ID:
C3808145
•
Disease or Syndrome
Synonym: LVNC9
 
Monarch Initiative: MONDO:0800346
OMIM®: 191010; 611878

Professional guidelines

PubMed

Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free PMC Article
Genetic Testing in Pediatric Left Ventricular Noncompaction.
Miller EM, Hinton RB, Czosek R, Lorts A, Parrott A, Shikany AR, Ittenbach RF, Ware SM
Circ Cardiovasc Genet 2017 Dec;10(6) doi: 10.1161/CIRCGENETICS.117.001735. PMID: 29212898Free PMC Article
Differential diagnoses and extracardiac manifestations in left ventricular noncompaction.
Stöllberger C, Finsterer J
J Cardiovasc Med (Hagerstown) 2007 Sep;8(9):721. doi: 10.2459/JCM.0b013e32803cab3a. PMID: 17700404

Recent clinical studies

Etiology

Role of TBX20 Truncating Variants in Dilated Cardiomyopathy and Left Ventricular Noncompaction.
Amor-Salamanca A, Santana Rodríguez A, Rasoul H, Rodríguez-Palomares JF, Moldovan O, Hey TM, Delgado MG, Cuenca DL, de Castro Campos D, Basurte-Elorz MT, Macías-Ruiz R, Fuentes Cañamero ME, Galvin J, Bilbao Quesada R, de la Higuera Romero L, Trujillo-Quintero JP, García-Cruz LM, Cárdenas-Reyes I, Jiménez-Jáimez J, García-Hernández S, Valverde-Gómez M, Gómez-Díaz I, Limeres Freire J, García-Pinilla JM, Gimeno-Blanes JR, Savattis K, García-Pavía P, Ochoa JP
Circ Genom Precis Med 2024 Apr;17(2):e004404. Epub 2024 Feb 14 doi: 10.1161/CIRCGEN.123.004404. PMID: 38353104Free PMC Article
Stroke and embolism in patients with left ventricular hypertrabeculation/noncompaction.
Schiebel K, Finsterer J, Lazarevic P, Stöllberger C
J Stroke Cerebrovasc Dis 2022 Sep;31(9):106623. Epub 2022 Jul 6 doi: 10.1016/j.jstrokecerebrovasdis.2022.106623. PMID: 35803121
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
Left Ventricular Non-Compaction in Athletes: To Play or Not to Play.
Coris EE, Moran BK, De Cuba R, Farrar T, Curtis AB
Sports Med 2016 Sep;46(9):1249-59. doi: 10.1007/s40279-016-0512-2. PMID: 27002622
Role of cardiac MRI in the assessment of nonischemic cardiomyopathies.
Kassi M, Nabi F
Methodist Debakey Cardiovasc J 2013 Jul-Sep;9(3):149-55. doi: 10.14797/mdcj-9-3-149. PMID: 24066198Free PMC Article

Diagnosis

Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free 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
Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?
Arbustini E, Favalli V, Narula N, Serio A, Grasso M
J Am Coll Cardiol 2016 Aug 30;68(9):949-66. doi: 10.1016/j.jacc.2016.05.096. PMID: 27561770
Role of cardiac MRI in the assessment of nonischemic cardiomyopathies.
Kassi M, Nabi F
Methodist Debakey Cardiovasc J 2013 Jul-Sep;9(3):149-55. doi: 10.14797/mdcj-9-3-149. PMID: 24066198Free PMC Article
Left ventricular trabecular non-compaction.
Patel KC, Pennell D, Leyva-Leon F
Heart 2004 Sep;90(9):1076. doi: 10.1136/hrt.2003.032888. PMID: 15310714Free PMC Article

Therapy

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy.
Chimenti C, Lavalle C, Magnocavallo M, Alfarano M, Mariani MV, Bernardini F, Della Rocca DG, Galardo G, Severino P, Di Lullo L, Miraldi F, Fedele F, Frustaci A
ESC Heart Fail 2022 Feb;9(1):241-250. Epub 2021 Dec 16 doi: 10.1002/ehf2.13694. PMID: 34918480Free 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
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
Implantable cardioverter defibrillator in a young child with left ventricular noncompaction.
Amadieu R, Acar P, Séguéla PE
Arch Cardiovasc Dis 2011 Jun-Jul;104(6-7):417-8. Epub 2011 May 31 doi: 10.1016/j.acvd.2010.10.011. PMID: 21798475

Prognosis

Stroke and embolism in patients with left ventricular hypertrabeculation/noncompaction.
Schiebel K, Finsterer J, Lazarevic P, Stöllberger C
J Stroke Cerebrovasc Dis 2022 Sep;31(9):106623. Epub 2022 Jul 6 doi: 10.1016/j.jstrokecerebrovasdis.2022.106623. PMID: 35803121
Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free 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
Left Ventricular Noncompaction in Older Patients.
Tian T, Yang KQ, Mao Y, Zhou LL, Wang LP, Xiao Y, Yang YK, Zhang Y, Meng X, Zhou XL
Am J Med Sci 2017 Aug;354(2):140-144. Epub 2017 Apr 21 doi: 10.1016/j.amjms.2017.04.012. PMID: 28864371
Left Ventricular Non-Compaction in Athletes: To Play or Not to Play.
Coris EE, Moran BK, De Cuba R, Farrar T, Curtis AB
Sports Med 2016 Sep;46(9):1249-59. doi: 10.1007/s40279-016-0512-2. PMID: 27002622

Clinical prediction guides

Role of TBX20 Truncating Variants in Dilated Cardiomyopathy and Left Ventricular Noncompaction.
Amor-Salamanca A, Santana Rodríguez A, Rasoul H, Rodríguez-Palomares JF, Moldovan O, Hey TM, Delgado MG, Cuenca DL, de Castro Campos D, Basurte-Elorz MT, Macías-Ruiz R, Fuentes Cañamero ME, Galvin J, Bilbao Quesada R, de la Higuera Romero L, Trujillo-Quintero JP, García-Cruz LM, Cárdenas-Reyes I, Jiménez-Jáimez J, García-Hernández S, Valverde-Gómez M, Gómez-Díaz I, Limeres Freire J, García-Pinilla JM, Gimeno-Blanes JR, Savattis K, García-Pavía P, Ochoa JP
Circ Genom Precis Med 2024 Apr;17(2):e004404. Epub 2024 Feb 14 doi: 10.1161/CIRCGEN.123.004404. PMID: 38353104Free PMC Article
Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free 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
Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?
Arbustini E, Favalli V, Narula N, Serio A, Grasso M
J Am Coll Cardiol 2016 Aug 30;68(9):949-66. doi: 10.1016/j.jacc.2016.05.096. PMID: 27561770
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

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
Thromboembolic events in left ventricular non-compaction: comparison between children and adults - a systematic review and meta-analysis.
Hirono K, Takarada S, Miyao N, Nakaoka H, Ibuki K, Ozawa S, Origasa H, Ichida F
Open Heart 2022 May;9(1) doi: 10.1136/openhrt-2021-001908. PMID: 35613828Free 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
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
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

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

    Recent activity