Abstract
Left ventricular non-compaction (LVNC) is thought to arise from arrest of the normal process of trabecular remodeling or “compaction” that takes place during embryonic life and is characterized by the presence of a two-layered ventricular wall, with a compact epicardial layer and a non-compacted endocardial layer. It is an uncommon condition that can occur isolated or in association with other disorders, including congenital heart anomalies and mitochondrial or musculoskeletal disorders. Both familial and sporadic forms are recognized, and several responsible genes have been identified, although only a minority of patients can be successfully genotyped. The diagnosis is usually made by echocardiography, but cardiac magnetic resonance imaging has been used increasingly. Management is mainly empirical and directed at the major clinical manifestations: heart failure, arrhythmias, and systemic embolic events. This article will review the major features of LVNC and present new trends in the diagnosis and management of this intriguing condition.
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Abbreviations
- ICD:
-
Implantable cardioverter-defibrillator
- LGE:
-
Late gadolinium enhancement
- LV:
-
Left ventricle
- LVNC:
-
Left ventricular non-compaction
- CMR:
-
Cardiac magnetic resonance imaging
References
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Pedro Carrilho-Ferreira, Ana G. Almeida, and Fausto J. Pinto declare that they have no conflict of interest.
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Cine 4-chamber view from a transthoracic echocardiogram showing the typical two-layered myocardium, with prominent trabeculations and deep recesses involving the apical segments of both the septal and lateral walls and the mid-lateral segment (AVI 7.02 MB)
Cine zoomed 4-chamber view from a contrast echocardiogram demonstrating the presence of contrast as expressing blood flow in the myocardial recesses (AVI 12.1 MB)
Cine 2-chamber view from a cardiac magnetic resonance imaging showing non-compaction with the two-layered myocardium at the apical segments of both the inferior and anterior walls and the mid-anterior segment (AVI 2.89 MB)
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Carrilho-Ferreira, P., Almeida, A.G. & Pinto, F.J. Non-compaction Cardiomyopathy: Prevalence, Prognosis, Pathoetiology, Genetics, and Risk of Cardioembolism. Curr Heart Fail Rep 11, 393–403 (2014). https://doi.org/10.1007/s11897-014-0227-3
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DOI: https://doi.org/10.1007/s11897-014-0227-3