title: Impact of clinical and echocardiographic characteristics on occurrence of cardiac events in cardiac amyloidosis as proven by endomyocardial biopsy.
abstract: BACKGROUND
  Although patients with immunoglobulin light chain (AL) cardiac amyloidosis exhibit worse outcomes than those with transthyretin (TTR) cardiac amyloidosis, few data exist regarding the occurrence of cardiac events and the echocardiographic indices in endomyocardial biopsy (EMBx) proven amyloidosis.
  
  
  METHODS
  From November 2007 to October 2012, we identified 33 patients with EMBx-proven amyloidosis. There were 12 patients (8 men; mean age: 66 years) with AL and 21 patients (20 men; mean age: 78 years) with TTR. We performed serial echocardiography and observed the patients during follow-up; defining all-cause mortality as the primary endpoint and hospitalization for heart failure as the secondary endpoint.
  
  
  RESULTS
  The survival rates at 12 months were 20.8% and 85.7% in AL and TTR, respectively (p<0.001). The cumulative incidences of the composite of death or readmission for heart failure at 12 months were 91.7% and 51.3% in AL and TTR, respectively (p<0.001). A multivariate analysis showed that the AL type amyloid was the powerful predictor of mortality (hazard ratio: 8.50, 95% confidence interval: 1.79 to 40.57, p<0.05). Under these conditions, the E/e' in AL tended to increase from 23±13 to 28±11 (p=0.06) with marked increases in B-type natriuretic peptide (779±456 pg/ml to 1576±895 pg/ml, p<0.05), although these remained unchanged in TTR, which exhibited significantly increased left ventricular end-diastolic dimensions from 40±4 mm to 42±4 mm (p<0.05).
  
  
  CONCLUSIONS
  The survival rate was generally worse in AL cardiac amyloidosis, although the readmission for heart failure remains high in TTR cardiac amyloidosis with the occurrence of left ventricular dilatation.
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Nakahashi
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Arita
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Kyohei
authors.initials: K
authors.lastname: Yamaji
authors.affiliation: Department of Laboratory Medicine, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Katsumi
authors.initials: K
authors.lastname: Inoue
authors.affiliation: Department of Pathology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Tadaaki
authors.initials: T
authors.lastname: Yokota
authors.affiliation: Department of Pathology, Yamaguchi University School of Medicine, Ube, Japan.
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Hoshii
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Fukunaga
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Nomura
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Hirotoshi
authors.initials: H
authors.lastname: Watanabe
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Shirou
authors.initials: S
authors.lastname: Miura
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Isotani
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Yoshimitsu
authors.initials: Y
authors.lastname: Soga
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Ando
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Iwabuchi
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Yokoi
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Nosaka
authors.affiliation: Division of Cardiovascular Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan. Electronic address: myamagi@med.kanazawa-u.ac.jp.
authors.forename: Masakazu
authors.initials: M
authors.lastname: Yamagishi
authors.affiliation: Division of Cardiology, Kokura Memorial Hospital, Kitakyusyu, Japan.
authors.forename: Masakiyo
authors.initials: M
authors.lastname: Nobuyoshi
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000401:mortality / Q000473:pathology*; D001706:Biopsy / Q000379:methods; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology; D018619:Echocardiography, Doppler, Pulsed / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000000981:diagnostic imaging* / Q000401:mortality / Q000473:pathology*; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D015996:Survival Rate / Q000639:trends
keywords: Cardiac amyloidosis; Endomyocardial biopsy; Left ventricular function; Prognosis