[Amyloidosis of the heart].
Amyloidosis is a heterogeneous group of diseases characterized by the pathological deposition of autologous proteins in an antiparallel β-sheet confirmation forming non-branching linear fibrils of indefinite length and an approximate diameter of 10-12 nm. Cardiac amyloidosis is caused by deposits in the heart and may lead to cardiac arrhythmia and low output failure. Following the diagnosis, classification of the amyloid protein and evaluation of further organ involvement is mandatory. Treatment approaches are based on reduction of the production of amyloid precursor proteins. Standard heart failure treatment is usually not well tolerated and the underlying disease remains unaffected. Cardiac amyloidosis, especially of the light chain type, is associated with a poor outcome. The clinical picture is uncharacteristic, therefore correct diagnosis of cardiac amyloidosis is often delayed in many patients. Combination of clinical symptoms of different organ systems should alert the physician to the diagnosis of amyloidosis.
Abteilung für Innere Medizin III (Kardiologie, Angiologie, Pneumologie), Medizinische Universitätsklinik Heidelberg, Heidelberg, Deutschland.
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D000367:Age Factors; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000235:genetics / Q000473:pathology*; D009320:Atrial Natriuretic Factor / Q000378:metabolism; D001706:Biopsy; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000473:pathology*; D009206:Myocardium / Q000473:pathology; D011228:Prealbumin / Q000378:metabolism; D012042:Registries; D012737:Sex Factors
D000682:Amyloid; D011228:Prealbumin; C035222:amyloid protein AL; D009320:Atrial Natriuretic Factor