IMR Press / RCM / Volume 11 / Issue 2 / DOI: 10.3909/ricm0531

Reviews in Cardiovascular Medicine (RCM) is published by IMR Press from Volume 19 Issue 1 (2018). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with MedReviews, LLC.

Open Access Review
Left Ventricular Noncompaction Cardiomyopathy: What Do We Know?
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1 Division of Cardiovascular Disease, Mayo Clinic Florida, Jacksonville, FL
2 Division of Genetics, Mayo Clinic, Rochester, MN
3 Division of Cardiovascular Disease, Aurora Medical Center, Milwaukee, WI
Rev. Cardiovasc. Med. 2010, 11(2), 92–99; https://doi.org/10.3909/ricm0531
Published: 30 June 2010
Abstract
Noncompaction is a rare and primary genetic cardiomyopathy affecting the left ventricle. The diagnosis is usually established by echocardiography, or, less frequently, by left ventriculography. Cardiac magnetic resonance and electrocardiography-gated multidetector computed tomography are evolving noninvasive modalities to image cardiac structures, and have the utility to detect noncompacted myocardium. Estimates of the frequency and incidence of left ventricular noncompaction (LVNC) are limited because of the controversy over whether LVNC is a discrete disease entity. There is considerable overlap with dilated cardiomyopathy, apical hypertrophy, and hypertrophic cardiomyopathy. Symptoms, diagnosis, and prognosis are variable because of the heterogeneous nature of these diseases, making treatment often empirical and mimicking the treatment of other cardiomyopathies. However, there are management issues that should be addressed in each patient with LVNC, including genetic testing and family screening, the need for implantable cardioverter defibrillator placement, the role of anticoagulation in prevention of thromboembolic complications, and prescriptions/restrictions for implementation of physical activity.
Keywords
Noncompaction
Cardiomyopathy
Hypertrabeculation
Automatic implantable cardioverter defibrillator
Thromboembolism
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