isolated noncompaction of the ventricular myocardium


Summary: 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.

Top Publications

  1. Chaowu Y, Li L, Shihua Z. Histopathological features of delayed enhancement cardiovascular magnetic resonance in isolated left ventricular noncompaction. J Am Coll Cardiol. 2011;58:311-2 pubmed publisher
  2. Attenhofer Jost C, Connolly H. Left ventricular non-compaction: dreaming of the perfect diagnostic tool. Eur J Heart Fail. 2012;14:113-4 pubmed publisher
  3. Cao Q, Shen Y, Liu X, Yu X, Yuan P, Wan R, et al. Phenotype and Functional Analyses in a Transgenic Mouse Model of Left Ventricular Noncompaction Caused by a DTNA Mutation. Int Heart J. 2017;: pubmed publisher
    ..In conclusion, overexpression of the DTNA-p.N49S mutation in a mouse heart can be responsible for the phenotype of deep trabeculation, dilated cardiomyopathy, and cardiac dysfunction, which resembles the phenotype of LVNC...
  4. Gandhi R, Sarraf G, Budoff M. Isolated noncompaction of the left ventricular myocardium diagnosed upon cardiovascular multidetector computed tomography. Tex Heart Inst J. 2010;37:374-5 pubmed
  5. Poscolieri B, Bianco M, Vessella T, Gervasi S, Palmieri V, Zeppilli P. Identification of benign form of ventricular non-compaction in competitive athletes by multiparametric evaluation. Int J Cardiol. 2014;176:1134-6 pubmed publisher
  6. Hastings R, de Villiers C, Hooper C, Ormondroyd L, Pagnamenta A, Lise S, et al. Combination of Whole Genome Sequencing, Linkage, and Functional Studies Implicates a Missense Mutation in Titin as a Cause of Autosomal Dominant Cardiomyopathy With Features of Left Ventricular Noncompaction. Circ Cardiovasc Genet. 2016;9:426-435 pubmed
    ..It furthermore highlights that rare titin missense variants, currently often ignored or left uninterpreted, should be considered to be relevant for cardiomyopathies and can be identified by the approach presented here. ..
  7. Ivanov A, Dabiesingh D, Bhumireddy G, Mohamed A, Asfour A, Briggs W, et al. Prevalence and Prognostic Significance of Left Ventricular Noncompaction in Patients Referred for Cardiac Magnetic Resonance Imaging. Circ Cardiovasc Imaging. 2017;10: pubmed publisher
    ..The diagnosis of LVNC, by any current criteria, was not associated with adverse clinical events on nearly 7 years of follow-up. Limited conclusions can be made for Captur criteria due to low observed prevalence. ..
  8. Towbin J, Jefferies J. Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism. Circ Res. 2017;121:838-854 pubmed publisher
    ..In addition, cardiomyopathies resulting from mitochondrial dysfunction, metabolic abnormalities, storage diseases, and inborn errors of metabolism are described. ..