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Left ventricular non-compaction: Genetic heterogeneity, diagnosis and clinical course

Captur, G; Nihoyannopoulos, P; (2010) Left ventricular non-compaction: Genetic heterogeneity, diagnosis and clinical course. International Journal of Cardiology , 140 (2) pp. 145-153. 10.1016/j.ijcard.2009.07.003. Green open access

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Abstract

Left ventricular non-compaction (LVNC) is a rare disorder that results in multiple deep trabeculations within the left ventricular myocardium. It is thought to be due in part, to an arrest of myocardial development but more recent evidence suggests that some cases may actually be acquired while other isolated cases have regressed with time. Transthoracic echocardiography remains the imaging modality of choice for LVNC where diagnosis is based on the identification of multiple prominent ventricular trabeculations with intertrabecular spaces communicating with the ventricular cavity. There is a broad and potentially confusing spectrum of clinical symptomatology in patients with ventricular non-compaction meaning that the primary diagnosis is often missed. Complications such as potentially malignant arrhythmias, left ventricular failure, and cardioembolic events arising as a result of non-compaction must be treated in an attempt to decrease morbidity and mortality from this disorder. The ultimate outcome for patients remains unclear with some boasting a prolonged asymptomatic course, to others displaying a rapid deterioration of left ventricular systolic function, leading to heart transplantation or death. In conclusion, LVNC while remaining a rare cardiomyopathy, shall probably be diagnosed with increasing frequency in the coming years because of heightened awareness about its natural history and clinical manifestations and because of the improved modalities available for cardiac imaging. © 2009 Elsevier Ireland Ltd. All rights reserved.

Type: Article
Title: Left ventricular non-compaction: Genetic heterogeneity, diagnosis and clinical course
Location: Netherlands
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ijcard.2009.07.003
Publisher version: https://doi.org/10.1016/j.ijcard.2009.07.003
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Cardiomyopathy, Echocardiography, Heart Failure
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Population Science and Experimental Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Population Science and Experimental Medicine > MRC Unit for Lifelong Hlth and Ageing
URI: https://discovery-pp.ucl.ac.uk/id/eprint/10199500
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