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Abnormal morphology of myocardial trabeculae

MedGen UID:
892696
•Concept ID:
C4073289
•
Anatomical Abnormality
HPO: HP:0030681

Definition

Any structural anomaly of the muscular columns which project from the inner surface of the right and left ventricles of the heart (cardiac trabeculae, trabeculae carneae). [from HPO]

Recent clinical studies

Etiology

Late gadolinium enhancement on CMRI in patients with LV noncompaction: An overestimated phenomenon?
Asmakutlu O, Alis D, Topel C, Sahin A
Clin Imaging 2020 Oct;66:121-126. Epub 2020 May 4 doi: 10.1016/j.clinimag.2020.04.024. PMID: 32480266
Nkx2-5 and Sarcospan genetically interact in the development of the muscular ventricular septum of the heart.
Panzer AA, Regmi SD, Cormier D, Danzo MT, Chen ID, Winston JB, Hutchinson AK, Salm D, Schulkey CE, Cochran RS, Wilson DB, Jay PY
Sci Rep 2017 Apr 13;7:46438. doi: 10.1038/srep46438. PMID: 28406175Free PMC Article
Feasibility of ferumoxytol-enhanced neonatal and young infant cardiac MRI without general anesthesia.
Lai LM, Cheng JY, Alley MT, Zhang T, Lustig M, Vasanawala SS
J Magn Reson Imaging 2017 May;45(5):1407-1418. Epub 2016 Sep 28 doi: 10.1002/jmri.25482. PMID: 27678106Free PMC Article
Quantification of left ventricular trabeculae using fractal analysis.
Captur G, Muthurangu V, Cook C, Flett AS, Wilson R, Barison A, Sado DM, Anderson S, McKenna WJ, Mohun TJ, Elliott PM, Moon JC
J Cardiovasc Magn Reson 2013 May 10;15(1):36. doi: 10.1186/1532-429X-15-36. PMID: 23663522Free PMC Article
Pathology and pathogenesis of arrhythmogenic right ventricular cardiomyopathy.
Thiene G, Basso C, Calabrese F, Angelini A, Valente M
Herz 2000 May;25(3):210-5. doi: 10.1007/s000590050008. PMID: 10904840

Diagnosis

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
Formation and Malformation of Cardiac Trabeculae: Biological Basis, Clinical Significance, and Special Yield of Magnetic Resonance Imaging in Assessment.
Captur G, Syrris P, Obianyo C, Limongelli G, Moon JC
Can J Cardiol 2015 Nov;31(11):1325-37. Epub 2015 Jul 8 doi: 10.1016/j.cjca.2015.07.003. PMID: 26440509
Abnormal cardiac formation in hypertrophic cardiomyopathy: fractal analysis of trabeculae and preclinical gene expression.
Captur G, Lopes LR, Patel V, Li C, Bassett P, Syrris P, Sado DM, Maestrini V, Mohun TJ, McKenna WJ, Muthurangu V, Elliott PM, Moon JC
Circ Cardiovasc Genet 2014 Jun;7(3):241-8. Epub 2014 Apr 5 doi: 10.1161/CIRCGENETICS.113.000362. PMID: 24704860
Noncompaction syndrome of the myocardium: pathophysiology and imaging pearls.
Zenooz NA, Zahka KG, Siwik ES, Gilkeson RC
J Thorac Imaging 2010 Nov;25(4):326-32. doi: 10.1097/RTI.0b013e31819ca93e. PMID: 20216462
Helical CT features of arrhythmogenic right ventricular cardiomyopathy.
Kimura F, Sakai F, Sakomura Y, Fujimura M, Ueno E, Matsuda N, Kasanuki H, Mitsuhashi N
Radiographics 2002 Sep-Oct;22(5):1111-24. doi: 10.1148/radiographics.22.5.g02se031111. PMID: 12235341

Therapy

Novel insights on the relationship between T-tubular defects and contractile dysfunction in a mouse model of hypertrophic cardiomyopathy.
Crocini C, Ferrantini C, Scardigli M, Coppini R, Mazzoni L, Lazzeri E, Pioner JM, Scellini B, Guo A, Song LS, Yan P, Loew LM, Tardiff J, Tesi C, Vanzi F, Cerbai E, Pavone FS, Sacconi L, Poggesi C
J Mol Cell Cardiol 2016 Feb;91:42-51. Epub 2015 Dec 20 doi: 10.1016/j.yjmcc.2015.12.013. PMID: 26714042Free PMC Article

Prognosis

Myofiber organization in the failing systemic right ventricle.
Campanale CM, Scherrer B, Afacan O, Majeed A, Warfield SK, Sanders SP
J Cardiovasc Magn Reson 2020 Jun 29;22(1):49. doi: 10.1186/s12968-020-00637-9. PMID: 32600420Free PMC Article
Nkx2-5 and Sarcospan genetically interact in the development of the muscular ventricular septum of the heart.
Panzer AA, Regmi SD, Cormier D, Danzo MT, Chen ID, Winston JB, Hutchinson AK, Salm D, Schulkey CE, Cochran RS, Wilson DB, Jay PY
Sci Rep 2017 Apr 13;7:46438. doi: 10.1038/srep46438. PMID: 28406175Free PMC Article
Prediction of sarcomere mutations in subclinical hypertrophic cardiomyopathy.
Captur G, Lopes LR, Mohun TJ, Patel V, Li C, Bassett P, Finocchiaro G, Ferreira VM, Esteban MT, Muthurangu V, Sherrid MV, Day SM, Canter CE, McKenna WJ, Seidman CE, Bluemke DA, Elliott PM, Ho CY, Moon JC
Circ Cardiovasc Imaging 2014 Nov;7(6):863-71. Epub 2014 Sep 16 doi: 10.1161/CIRCIMAGING.114.002411. PMID: 25228707Free PMC Article
Noncompaction syndrome of the myocardium: pathophysiology and imaging pearls.
Zenooz NA, Zahka KG, Siwik ES, Gilkeson RC
J Thorac Imaging 2010 Nov;25(4):326-32. doi: 10.1097/RTI.0b013e31819ca93e. PMID: 20216462
Primary noncompaction of the ventricular myocardium from the morphogenetic standpoint.
Bartram U, Bauer J, Schranz D
Pediatr Cardiol 2007 Sep-Oct;28(5):325-32. Epub 2007 Jul 12 doi: 10.1007/s00246-006-0054-8. PMID: 17632682

Clinical prediction guides

Late gadolinium enhancement on CMRI in patients with LV noncompaction: An overestimated phenomenon?
Asmakutlu O, Alis D, Topel C, Sahin A
Clin Imaging 2020 Oct;66:121-126. Epub 2020 May 4 doi: 10.1016/j.clinimag.2020.04.024. PMID: 32480266
Feasibility of ferumoxytol-enhanced neonatal and young infant cardiac MRI without general anesthesia.
Lai LM, Cheng JY, Alley MT, Zhang T, Lustig M, Vasanawala SS
J Magn Reson Imaging 2017 May;45(5):1407-1418. Epub 2016 Sep 28 doi: 10.1002/jmri.25482. PMID: 27678106Free 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
Prediction of sarcomere mutations in subclinical hypertrophic cardiomyopathy.
Captur G, Lopes LR, Mohun TJ, Patel V, Li C, Bassett P, Finocchiaro G, Ferreira VM, Esteban MT, Muthurangu V, Sherrid MV, Day SM, Canter CE, McKenna WJ, Seidman CE, Bluemke DA, Elliott PM, Ho CY, Moon JC
Circ Cardiovasc Imaging 2014 Nov;7(6):863-71. Epub 2014 Sep 16 doi: 10.1161/CIRCIMAGING.114.002411. PMID: 25228707Free PMC Article
Isolated left ventricular noncompaction syndrome.
Stanton C, Bruce C, Connolly H, Brady P, Syed I, Hodge D, Asirvatham S, Friedman P
Am J Cardiol 2009 Oct 15;104(8):1135-8. Epub 2009 Aug 28 doi: 10.1016/j.amjcard.2009.05.062. PMID: 19801037

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