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      Unusual Cardiomyopathies: Some May Be More Usual Than Previously Thought and Simply Underdiagnosed

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          Abstract

          Heart failure is the most common cause of hospitalization in the United States. Just as the prevalence of heart failure has increased, the number of diseases identified that result in the heart failure syndrome has escalated. Certain cardiomyopathies that have previously been regarded as very rare are being recognized with increasing frequency, because of improved imaging techniques and an increased understanding of the pathophysiologic mechanisms that result in these diseases. Improved echocardiographic techniques and methods such as spectral Doppler and 3D image rendering, along with the use of advanced diagnostic tools such as cardiac CT angiography and cardiac magnetic resonance imaging are now common. These advanced imaging methods have led to an increased appreciation of the frequency of diseases such as isolated left ventricular noncompaction and cardiac amyloidosis. Left ventricular noncompaction, once thought to occur in roughly one in one million patients, may actually be seen in fewer than one in 1000 patients. Cardiac amyloidosis, in the senile form, may exist in 80% of 80-year-old patients, although the incidence of clinical symptoms is less. As the genetic alterations that contribute to these diseases are further elucidated, improved diagnosis and a better understanding of the prognosis of these uncommon cardiomyopathies will follow.

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          Most cited references 28

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          Left ventricular noncompaction: a new form of heart failure.

          In this article the newly classified cardiomyopathy known as left ventricular noncompaction is discussed. This genetic inherited form of heart disease has substantial risk of heart failure, stroke, metabolic derangement, arrhythmias, and sudden cardiac death. The disorder seems to occur because of an arrest of the normal process of development, and the genes identified to date seem to encode for cytoskeletal or sarcomeric proteins. These features are outlined. Copyright © 2010. Published by Elsevier Inc.
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            An unusual anomaly of the coronary vessels in the malformed heart of a child

             T. GRANT,  R GRANT,  RT Grant (1926)
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              Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau a population-based autopsy study

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                Author and article information

                Journal
                CVIA
                Cardiovascular Innovations and Applications
                CVIA
                Compuscript (Ireland )
                2009-8618
                2009-8618
                October 2015
                October 2015
                : 1
                : 1
                : 29-36
                Affiliations
                1Louisiana State University Health Sciences Center, New Orleans, LA, USA
                Author notes
                Correspondence: Frank W. Smart, MD, FACC, FACP, LSUHSC Cardiology, 533 Bolivar St. CSRB 3-42, New Orleans, LA 70112, USA,E-mail: fsmart@ 123456LSUHSC.EDU
                Article
                cvia20150006
                10.15212/CVIA.2015.0006
                Copyright © 2015 Cardiovascular Innovations and Applications

                This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 Unported License (CC BY-NC 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See https://creativecommons.org/licenses/by-nc/4.0/.

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