Left ventricular noncompaction

Definition:

Left ventricular noncompaction (LVNC) is a rare and potentially progressive cardiomyopathy, characterized by the presence of prominent trabeculations of the left ventricle, associated with progressive systolic failure, stroke and arrhythmia. It has been linked to mutations in several genes, including LIM domain binding protein 3 (ZASP), alpha-dystrobrevin (DTNA), and genes encoding the sarcomeric proteins, beta-myosin heavy chain (MYH7), alpha-cardiac actin (ACTC), and cardiac troponin T (TNNT2).

References:

[1]. Aleksandra Nijak, et al. Left ventricular non-compaction with Ebstein anomaly attributed to a TPM1 mutation. Eur J Med Genet. 2018 Jan;61(1):8-10. [Content Brief]

[2]. Anne-Karin Arndt, et al. Fine mapping of the 1p36 deletion syndrome identifies mutation of PRDM16 as a cause of cardiomyopathy. Am J Hum Genet. 2013 Jul 11;93(1):67-77. [Content Brief]

[3]. Erwin Oechslin, et al. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity?. Eur Heart J. 2011 Jun;32(12):1446-56. [Content Brief]

[4]. Fukiko Ichida, et al. Left ventricular noncompaction. Circ J. 2009 Jan;73(1):19-26. [Content Brief]

[5]. Guillermo Luxán, et al. Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathy. Nat Med. 2013 Feb;19(2):193-201. [Content Brief]

[6]. Lorenzo Monserrat, et al. Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, and septal defects. Eur Heart J. 2007 Aug;28(16):1953-61. [Content Brief]

[7]. Mark Luedde, et al. Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Cardiovasc Res. 2010 Jun 1;86(3):452-60. [Content Brief]

[8]. Matteo Vatta, et al. Mutations in Cypher/ZASP in patients with dilated cardiomyopathy and left ventricular non-compaction. J Am Coll Cardiol. 2003 Dec 3;42(11):2014-27. [Content Brief]

[9]. Qing Cao, et al. Phenotype and Functional Analyses in a Transgenic Mouse Model of Left Ventricular Noncompaction Caused by a DTNA Mutation. Int Heart J. 2017 Dec 12;58(6):939-947. [Content Brief]

[10]. Sabine Klaassen, et al. Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation. 2008 Jun 3;117(22):2893-901. [Content Brief]

[11]. Susanne Probst, et al. Sarcomere gene mutations in isolated left ventricular noncompaction cardiomyopathy do not predict clinical phenotype. Circ Cardiovasc Genet. 2011 Aug 1;4(4):367-74. [Content Brief]

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