Left ventricular noncompaction cardiomyopathy: cardiac, neuromuscular, and genetic factors
- 12 January 2017
- journal article
- review article
- Published by Springer Science and Business Media LLC in Nature Reviews Cardiology
- Vol. 14 (4), 224-237
- https://doi.org/10.1038/nrcardio.2016.207
Abstract
Left ventricular hypertrabeculation (LVHT) or noncompaction is a myocardial abnormality of unknown aetiology, frequently associated with monogenic disorders, particularly neuromuscular disorders, or with chromosomal defects. LVHT is diagnosed usually by echocardiography by the presence of a bilayered myocardium consisting of a thick, spongy, noncompacted endocardial layer and a thin, compacted, epicardial layer. The pathogenesis of LVHT is unsolved, and the diagnostic criteria, prognosis, and optimal treatment of patients with LVHT are under debate. LVHT is categorized as distinct primary genetic cardiomyopathy by the AHA and as unclassified cardiomyopathy by the ESC. LVHT is usually asymptomatic, but can be complicated by heart failure, thromboembolism, or ventricular arrhythmias, including sudden cardiac death. Mortality of patients with LVHT ranges from 5% to 47%. Anticoagulation is indicated if atrial fibrillation, severe heart failure, previous embolism, or intracardiac thrombus formation are present. In patients with LVHT with late gadolinium enhancement, an implantable cardioverter–defibrillator might be considered if systolic dysfunction, a family history of sudden cardiac death, nonsustained ventricular tachycardia, or previous syncope is additionally present. In this Review, we discuss the current findings on the aetiology and pathophysiology of LVHT, and provide an overview of the diagnosis, available treatment, and prognosis of this cardiomyopathy.Keywords
This publication has 167 references indexed in Scilit:
- Late profound muscle weakness following heart transplantation due to danon diseaseMuscle & Nerve, 2012
- Noncompaction cardiomyopathy, a frequently overlooked entity (…but beware of over diagnosis!)Netherlands Heart Journal, 2012
- Novel desmoplakin mutation: juvenile biventricular cardiomyopathy with left ventricular non-compaction and acantholytic palmoplantar keratodermaClinical Research in Cardiology, 2011
- Regional Thicknesses and Thickening of Compacted and Trabeculated Myocardial Layers of the Normal Left Ventricle Studied by Cardiovascular Magnetic ResonanceCirculation: Cardiovascular Imaging, 2011
- Dishevelled-associated activator of morphogenesis 1 (Daam1) is required for heart morphogenesisDevelopment, 2011
- Subendocardial Fibrosis in Left Ventricular Hypertrabeculation-Cause or Consequence?Clinical Medicine Insights: Cardiology, 2011
- Isolated Left Ventricular Noncompaction Cardiomyopathy Diagnosed by Transesophageal EchocardiographyClinical Medicine Insights: Cardiology, 2011
- Embryogenesis of the Heart MuscleHeart Failure Clinics, 2008
- Acquired left ventricular noncompaction as a cardiac manifestation of neuromuscular disordersScandinavian Cardiovascular Journal, 2008
- Noncompaction of the Ventricular Myocardium Is Associated with a De Novo Mutation in the β-Myosin Heavy Chain GenePLOS ONE, 2007