Metabolic Cardiomyopathy, Second Edition

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  • Cardiomyopathy
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  • Hypertrophic cardiomyopathy - an overview | ScienceDirect Topics
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    • Cardiomyopathy

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    What is cardiomyopathy?

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    East Dane Designer Men's Fashion. Shopbop Designer Fashion Brands. The result is heightened clinical surveillance and possibly earlier intervention and prevention of the sequelae of cardiomyopathies. This may allow family members that carry the same genetic mutation to be followed, since they may have a reduced penetrance that leads to a lesser form of the disease or even asymptomatic expression.

    Most inherited cardiomyopathies demonstrate a mendelian autosomal dominant inheritance.

    Hypertrophic cardiomyopathy - an overview | ScienceDirect Topics

    It has been accepted that HCM is largely a genetic defect of the contractile proteins. The more common sporadic DCM has not been found to have a genetic basis.

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    Desmosomes, cell-cell adhesion organelles, are especially abundant in heart tissue. A genetic basis for restrictive cardiomyopathy RCM has not been identified, but a familial form exists and is caused by mutations in the troponin I gene. More extensive information about the genetics of primary cardiomyopathies is available. The cardiomyopathies are a heterogeneous group of disorders of cardiac muscle Table In some circumstances, mixed phenotypes can exist.

    For example, patients with hypertrophic and dilated cardiomyopathies frequently have restrictive left ventricular physiology; in other cases, hypertrophy and ventricular dilation may co-exist. Giordano, in Cardiovascular Pathology Fourth Edition , Cardiomyopathies with a hypertrophic phenotype have in common the genetic etiology.

    However, the pathways leading to the phenotypic expression and the clinical and morphologic features vary widely, according to the gene or class of genes involved.

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    Clues in the differential diagnosis include the pattern of inheritance, age of onset, and clinical presentation syndromic vs. The infiltrative cardiomyopathies include sarcoidosis, amyloidosis, and hemochromatosis.

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    Since infiltration of the tissue is accompanied by changes of myocardial signal properties, CMR may become a powerful diagnostic tool, although the specificity may be limited. Histologic findings are granulomas, which often are transmural. Probably because of the patchy distribution, however, the sensitivity of endomyocardial biopsy is low. Sarcoid heart disease may be accompanied by LV dilation as well as regional and global wall motion abnormalities, which can be more sensitively detected by CMR than with other modalities Regional granulomatous inflammation may lead to regional wall thinning and akinesis.

    Therefore, a careful functional and morphologic assessment by SSFP sequences, using a stack of contiguous slices, is recommended.

    Sarcoid lesions may lead to a range of signal intensities, possibly because of different stages of disease activity. Muscular sarcoidosis causes high signal intensity areas on T2-weighted CMR. In another study of skeletal muscle sarcoidosis, the granulomatous nodules exhibited a central region with low signal intensity in T1- and T2-weighted imaging but were surrounded by a high signal ring. Gadolinium accumulates in sarcoid lesions of the brain. This behavior may be compatible with fibrotic, not active granulomatous nodules with inflammatory response of the surrounding tissue.

    Similar findings were reported in several cases of sarcoid infiltration of the heart. T1-weighted imaging with assessment of early enhancement has been reported, and recently, LGE was reported in sarcoid heart disease. A combined protocol including a T2-weighted triple-inversion recovery spin echo sequence, a T1-weighted spin echo technique in short and long axis before and after a standard dose of gadolinium 0.

    In addition, the CMR findings could be used to guide the location for endomyocardial biopsy. Since the follow-up is very important to guide therapy, serial CMR studies may be very helpful in these patients.

    The World Health Organization defines cardiomyopathies CMs as myocardial diseases associated with cardiac dysfunction. They are classified by the dominant pathophysiology or, if known, by causative factors. Thus, CM may be dilated, hypertrophic, restrictive, or a special type called arrhythmogenic right ventricular cardiomyopathy or arrhythmogenic right ventricular dysplasia.

    If the cause of a CM is known i. CMs can also be associated with systemic disease, neuromuscular disorders, or exposure to toxins. Cardiomyopathies are disorders of heart muscle function. In some cases, these cardiomyopathies are associated with skeletal muscle dysfunction, and these overlapping phenotypes are called cardioskeletal myopathies.

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    The etiologies of these cardioskeletal myopathies include cytoskeletal, sarcomeric, nuclear membrane, and intermediate proteins. In addition, these cardioskeletal myopathies are associated with a variety of metabolic abnormalities, such as mitochondrial dysfunction and fatty acid oxidation disorders. Further, some cases may be associated with chromosomal defects or syndromes. In this chapter, we will 1 outline the clinical phenotypes of the classified cardiomyopathies including dilated cardiomyopathy , hypertrophic cardiomyopathy , restrictive cardiomyopathy , noncompaction cardiomyopathy , and arrhythmogenic cardiomyopathy that are associated with skeletal muscle abnormalities and 2 the etiologies causing these cardioskeletal myopathies.