The functional defect in amyloid heart disease

Clinical profile of restrictive cardiomyopathy.

“…In the few cases that have been reported, the prognosis has been uniformly poor. 6 In other instances, this clinical and hemodynamic profile may be In our series, patients with restrictive cardiomyopathy presented with symptoms of left and/or right ventricular failure, chest pain in the absence of significant narrowing of any epicardial coronary artery and/or symptoms from atrial arrhythmias. Physical findings were nonspecific, and evidence of systemic venous congestion was present in fewer than half the cases.…”

Evidence for Cardiomyopathy in Familial Diabetes Mellitus

“…After the onset of decompensation the diabetic myocardium presumably exhibits properties similar to other etiologic types. Altered interstitium may be the predominant lesion in the incipient stages of amyloid heart disease where end-diastolic pressure rather than volume elevations are characteristic (58). A similar, although less pronounced, pressure-volume abnormality may exist in diabetes.…”

“…Thus, the change in muscle function suggestive of enhanced wall stiffness may be related to altered composition in the form of interstitial glycoprotein and collagen accumulation observed in the morphologic studies. Extracellular structures are major determinants of muscle elasticity (57), and the observations in these diabetics are analogous to the amyloid heart where deposits between cardiac muscle cells enhance wall stiffness (58). In addition, in a diabetic animal model in which myofiber cell structure was normal, diminished end-diastolic compliance was also observed, without hypertrophy or altered indices of contractility (4).…”

Echocardiographic Assessment of the Cardiac Amyloidoses

“…6 The authors suggested that normal left ventricular (LV) dimensions in diastole, diminished amplitude of excursion, increased systolic dimensions, and pericardial effusion were strongly suggestive of cardiac amyloidosis. Child et al reported on the basic echocardiographic findings in infiltrative cardiomyopathy caused by amyloid, namely (1) symmetrically increased LV wall thickness in the absence of hypertension or aortic valvular disease, (2) hypokinesia and decreased systolic thickening of the interventricular septum and LV posterior wall, and (3) small-to-normal size of the LV cavity.…”