PMID- 23608605
OWN - NLM
STAT- MEDLINE
DCOM- 20130920
LR  - 20220317
IS  - 2047-9980 (Electronic)
IS  - 2047-9980 (Linking)
VI  - 2
IP  - 2
DP  - 2013 Apr 22
TI  - Senile systemic amyloidosis: clinical features at presentation and outcome.
PG  - e000098
LID - 10.1161/JAHA.113.000098 [doi]
LID - e000098
AB  - BACKGROUND: Cardiac amyloidosis is a fatal disease whose prognosis and treatment 
      rely on identification of the amyloid type. In our aging population transthyretin 
      amyloidosis (ATTRwt) is common and must be differentiated from other amyloid 
      types. We report the clinical presentation, natural history, and prognostic 
      features of ATTRwt compared with cardiac-isolated AL amyloidosis and calculate 
      the probability of disease diagnosis of ATTRwt from baseline factors. METHODS AND 
      RESULTS: All patients with biopsy-proven ATTRwt (102 cases) and isolated cardiac 
      AL (36 cases) seen from 2002 to 2011 at the UK National Amyloidosis Center were 
      included. Median survival from the onset of symptoms was 6.07 years in the ATTRwt 
      group and 1.7 years in the AL group. Positive troponin, a pacemaker, and 
      increasing New York Heart Association (NYHA) class were associated with worse 
      survival in ATTRwt patients on univariate analysis. All patients with isolated 
      cardiac AL and 24.1% of patients with ATTRwt had evidence of a plasma cell 
      dyscrasia. Older age and lower N-terminal pro-B-type natriuretic peptide (NT 
      pro-BNP) were factors significantly associated with ATTRwt. Patients aged 70 
      years and younger with an NT pro-BNP <183 pmol/L were more likely to have ATTRwt, 
      as were patients older than 70 years with an NT pro-BNP <1420 pmol/L. 
      CONCLUSIONS: Factors at baseline associated with a worse outcome in ATTRwt are 
      positive troponin T, a pacemaker, and NYHA class IV symptoms. The age of the 
      patient at diagnosis and NT pro-BNP level can aid in distinguishing ATTRwt from 
      AL amyloidosis.
FAU - Pinney, Jennifer H
AU  - Pinney JH
AD  - National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK. 
      j.pinney@ucl.ac.uk
FAU - Whelan, Carol J
AU  - Whelan CJ
FAU - Petrie, Aviva
AU  - Petrie A
FAU - Dungu, Jason
AU  - Dungu J
FAU - Banypersad, Sanjay M
AU  - Banypersad SM
FAU - Sattianayagam, Prayman
AU  - Sattianayagam P
FAU - Wechalekar, Ashutosh
AU  - Wechalekar A
FAU - Gibbs, Simon D J
AU  - Gibbs SD
FAU - Venner, Christopher P
AU  - Venner CP
FAU - Wassef, Nancy
AU  - Wassef N
FAU - McCarthy, Carolyn A
AU  - McCarthy CA
FAU - Gilbertson, Janet A
AU  - Gilbertson JA
FAU - Rowczenio, Dorota
AU  - Rowczenio D
FAU - Hawkins, Philip N
AU  - Hawkins PN
FAU - Gillmore, Julian D
AU  - Gillmore JD
FAU - Lachmann, Helen J
AU  - Lachmann HJ
LA  - eng
GR  - FS/09/063/28026/BHF_/British Heart Foundation/United Kingdom
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130422
PL  - England
TA  - J Am Heart Assoc
JT  - Journal of the American Heart Association
JID - 101580524
RN  - 0 (Amyloid)
RN  - 0 (Peptide Fragments)
RN  - 0 (Prealbumin)
RN  - 0 (pro-brain natriuretic peptide (1-76))
RN  - 114471-18-0 (Natriuretic Peptide, Brain)
SB  - IM
MH  - Aged
MH  - Amyloid/*classification/genetics
MH  - Amyloidosis/complications/*diagnosis/mortality
MH  - Biopsy
MH  - Cardiomyopathies/*diagnosis/etiology/mortality
MH  - Disease Progression
MH  - Echocardiography
MH  - Electrocardiography
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Myocardium/*metabolism/pathology
MH  - Natriuretic Peptide, Brain/blood
MH  - Peptide Fragments/blood
MH  - Prealbumin
MH  - Prognosis
MH  - Referral and Consultation/statistics & numerical data
PMC - PMC3647259
EDAT- 2013/04/24 06:00
MHDA- 2013/09/21 06:00
PMCR- 2013/04/01
CRDT- 2013/04/24 06:00
PHST- 2013/04/24 06:00 [entrez]
PHST- 2013/04/24 06:00 [pubmed]
PHST- 2013/09/21 06:00 [medline]
PHST- 2013/04/01 00:00 [pmc-release]
AID - 2/2/e000098 [pii]
AID - jah3193 [pii]
AID - 10.1161/JAHA.113.000098 [doi]
PST - epublish
SO  - J Am Heart Assoc. 2013 Apr 22;2(2):e000098. doi: 10.1161/JAHA.113.000098.