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Assessment of relative sensitivities of noninvasive tests for cardiac amyloidosis in documented cardiac amyloidosis

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    High LV filling pressures lead to significant postcapillary pulmonary hypertension and this may result in right ventricular cavity enlargement. As amyloid deposition occurs in the whole heart, thickened valves and interatrial septum may be noted in some patients, especially in more advanced disease [62]. The thickening of valve leaflets does not lead to hemodynamically significant regurgitant lesions [12].

  • Amyloidosis. Also a heart disease

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  • Relation of clinical, echocardiographic and electrocardiographic features of cardiac amyloidosis to the presence of the transthyretin V122I allele in older African-American men

    2011, American Journal of Cardiology
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    Table 4 lists the echocardiographic features of cardiac amyloidosis in the 2 groups. The initial association of a valine-to-isoleucine amino acid substitution in the serum protein TTR (TTR V122I) was noted in the amyloid fibrils extracted from the heart of an elderly African American man and confirmed in 2 additional probands.7–11 Biophysical studies showed that the mutant protein was less kinetically stable than wild-type TTR, accounting for its propensity to form amyloid.12

  • Evaluation and Management of the Cardiac Amyloidosis

    2007, Journal of the American College of Cardiology
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    Thus, although increased echogenicity is common in amyloid, its usefulness as a discriminating factor is limited. Reduced systolic function, as measured by low ejection fraction or poor wall thickening, is uncommon until the more severe stages of disease and can be absent in up to 75% of cases (12,23,25–27,33). Diastolic dysfunction is the hallmark, and may be present in all patients, though its identification can be difficult with standard techniques, with 21% to 88% of patients showing a restrictive pattern on Doppler mitral inflow assessment (25,35,36).

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