| Summary: | Introduction: Cardiac amyloidosis is a disease where misfolded proteins precipitate in the myocardium which eventually leads to diastolic dysfunction and restrictive cardiomyopathy. The disease has a poor prognosis but treatment is available that can slow down the disease progression but can not reverse tissue damage that has already occurred. Limited knowledge of the disease and its prevalence in the Icelandic population exists. This nationwide study aimed to assess the prevalence of transthyretin cardiac amyloidosis (ATTR-CA) and amyloid light chain cardiac amyloidosis (AL-CA) in Iceland, the age distribution, how many patients have received treatment with ATTR medication, and the survival of ATTR-CA patients. Methods: The study used the ICE-CAR register which contains data on patients in Iceland diagnosed with cardiac amyloidosis from May 2013 to March 2024 (N=78). Baseline characteristics were compared between cardiac amyloidosis types (ATTR-CA, AL-CA, other and unknown) but other results were only calculated for ATTR-CA patients. Survival analysis was done using the Kaplan-Meier method. Results: The distribution between cardiac amyloidosis types was: ATTR-CA (n=65), AL-CA (n=6), other types (n=1), and unknown type (n=6). Of the ATTR-CA population, 92.3% were male. The median age of diagnosis in the ATTR-CA patients was 81.4 years [64.9, 93.8]. The distribution of ATTR-CA patients in NYHA classes was 56.9% in NYHA I-II, 41.5% in NYHA III-IV, and 1.5% with a missing NYHA class. All ATTR-CA patients who underwent bone scintigraphy had in Perugini grade 2 (33.8%) or grade 3 (43.1%) and over half of the patients had apical sparing on echocardiography. Of the ATTR-CA patients, 32.3% had received ATTR treatment. The median survival overall in the ATTR-CA population was 22.6 months [0.100, 77.0]. Discussion: This is the first scientific review that has been carried out on ATTR-CA in Iceland and it provides a detailed national overview. Icelandic patients were on average significantly older and had more advanced ...
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