Prevalence of wild type transtyrethin cardiac amyloidosis in a heart failure clinic

Krister Lindmark, Björn Pilebro, Torbjörn Sundström, Per Lindqvist, Krister Lindmark, Björn Pilebro, Torbjörn Sundström, Per Lindqvist

Abstract

Aims: Wild type transthyretin amyloidosis (ATTRwt) has gained interest during recent years due to better diagnostic tools and the emergence of treatment options. Little is known about the prevalence of the disease. We aimed to investigate the prevalence in a heart failure population with myocardial hypertrophy.

Methods and results: All patients with an ICD code of heart failure living within the catchment area of Umeå University hospital and intraventricular septum >14 mm were offered screening with 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scan and a clinical work up. Out of 2238 patients with heart failure, 174 patients were found to have a septum >14 mm. Ten patients were already diagnosed with hereditary ATTR cardiomyopathy, 12 patients had ATTRwt cardiomyopathy, 12 patients had known HCM, one patient had AL amyloidosis, and four patients had already undergone a negative DPD scan (DPD uptake grade 0 and 1) within the last 3 years. This left 134 patients who we tried to contact for screening, but 48 patients had either died or declined to participate. Out of 86 screened patients, 13 had a DPD uptake of grade 2 or 3 without other amyloid disease making the total number of patients with ATTRwt in this population 25.

Conclusions: Approximately 20% of investigated patients in a cohort with heart failure and increased myocardial wall thickness has ATTRwt. Calculated for the whole population of heart failure patients, the prevalence is just over 1.1%. Comparing this number to the total population would give an estimated prevalence of 1:6000.

Keywords: ATTRwt; Amyloidosis; Cardiac amyloidosis; Heart failure; Transthyretin amyloidosis.

Conflict of interest statement

All authors declare no conflicts of interest regarding this study.

© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

Figures

Figure 1
Figure 1
Flow chart for patient cohort describing patient selection, drop out, and results. AL, light chain amyloid; ATTRv, hereditary amyloid; ATTRwt, wild type amyloid; HCM, sarcomeric hypertrophic cardiomyopathy; HF, heart failure; IVSD, end‐diastolic interventricular septum thickness.

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