Open AccessSenile Systemic Amyloidosis: Clinical Features at Presentation and Outcome
Author(s) -
Pinney Jennifer H.,
Whelan Carol J.,
Petrie Aviva,
Dungu Jason,
Banypersad Sanjay M.,
Sattianayagam Prayman,
Wechalekar Ashutosh,
Gibbs Simon D. J.,
Venner Christopher P.,
Wassef Nancy,
McCarthy Carolyn A.,
Gilbertson Janet A.,
Rowczenio Dorota,
Hawkins Philip N.,
Gillmore Julian D.,
Lachmann Helen J.
Publication year - 2013
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.113.000098
Subject(s) - medicine , amyloidosis , cardiac amyloidosis , natriuretic peptide , heart failure , population , univariate analysis , troponin , gastroenterology , dyscrasia , cardiology , multivariate analysis , myocardial infarction , environmental health , plasma cell , multiple myeloma
Background Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis ( ATTR wt) is common and must be differentiated from other amyloid types. We report the clinical presentation, natural history, and prognostic features of ATTR wt compared with cardiac‐isolated AL amyloidosis and calculate the probability of disease diagnosis of ATTR wt from baseline factors. Methods and Results All patients with biopsy‐proven ATTR wt (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 ATTR wt 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 ATTR wt patients on univariate analysis. All patients with isolated cardiac AL and 24.1% of patients with ATTR wt 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 ATTR wt. Patients aged 70 years and younger with an NT pro‐ BNP <183 pmol/L were more likely to have ATTR wt, 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 ATTR wt 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 ATTR wt from AL amyloidosis.