title: High prevalence of ATTR amyloidosis in endomyocardial biopsy-proven cardiac amyloidosis patients.
abstract: BACKGROUND
  Cardiac amyloidosis had been considered to be an incurable disease; however, new disease-modifying therapeutic approaches have succeeded in ameliorating the disease. Therefore, early and precise diagnosis based on the amyloid precursor protein is extremely important.
  
  
  OBJECTIVE
  To determine the prevalence rates of systemic amyloidoses underlying cardiac amyloidosis.
  
  
  METHODS
  The types of amyloidosis in 53 consecutive patients with endomyocardial biopsy-proven cardiac amyloidosis were analyzed by Congo red and immunohistochemical staining. If staining for TTR was positive, direct DNA sequencing of the entire TTR gene was performed.
  
  
  RESULTS
  ATTR amyloidosis was the most common (32/53 patients, 60.4%). The ATTR amyloidosis subtypes were senile systemic amyloidosis (SSA) 11, familial ATTR 10, and genotype unknown 11. AL amyloidosis was the next most frequent (19/53, 35.8%).
  
  
  CONCLUSIONS
  ATTR amyloidosis, especially SSA, might be much more common than previously thought. With the development of new drugs targeting the ATTR amyloidosis, major efforts should be made to increase awareness of senile systemic amyloidosis among cardiologists.
authors: Michitaka
authors: M
authors: Nakagawa
authors: Yoshiki
authors: Y
authors: Sekijima
authors: Kana
authors: K
authors: Tojo
authors: Shu-Ichi
authors: S
authors: Ikeda
mesh_terms: D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000453:epidemiology / Q000209:etiology* / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D005260:Female; D006331:Heart Diseases / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D016133:Polymerase Chain Reaction; D011228:Prealbumin / Q000235:genetics / Q000378:metabolism; D015995:Prevalence
chemical_list: D011228:Prealbumin