PT - JOURNAL ARTICLE AU - Rosa Elisabeth Lauppe AU - Johan Liseth Hansen AU - Christian Gerdesköld AU - Mark H Rozenbaum AU - Anne Mette Strand AU - Merja Vakevainen AU - Johanna Kuusisto AU - Einar Gude AU - Finn Gustafsson AU - J Gustav Smith TI - Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden AID - 10.1136/openhrt-2021-001755 DP - 2021 Oct 01 TA - Open Heart PG - e001755 VI - 8 IP - 2 4099 - http://openheart.bmj.com/content/8/2/e001755.short 4100 - http://openheart.bmj.com/content/8/2/e001755.full SO - Open Heart2021 Oct 01; 8 AB - Objective Transthyretin amyloid cardiomyopathy (ATTR-CM) is a rare, progressive and fatal condition caused by deposition of transthyretin amyloid fibrils in the heart. This study aims to identify all patients diagnosed with ATTR-CM in Sweden, estimate the prevalence of ATTR-CM, describe patient characteristics and mortality, assess the importance of early symptoms (red flags) for identification of ATTR-CM, and compare with patients with heart failure (HF).Methods This retrospective study combined multiple national health registers covering all specialist visits and prescriptions for the entire population of Sweden. Between January 2008 and December 2018, patients with ATTR-CM were identified retrospectively based on a combination of diagnosis codes and compared with matched, all-cause non-ATTR HF patients.Results Overall, a total of 994 patients diagnosed with ATTR-CM were identified, with an average age at diagnosis of 73 years, and 30% of whom were female. The prevalence of diagnosed ATTR-CM cases in 2018 was 5.0 per 100 000. The median survival from diagnosis was 37.6 months (CI 33.8 to 43.8), with a lower median survival in women (27.9 months, CI 23.3 to 33.8) compared with men (43.5 months, CI 37.6 to 49.6). Patients with ATTR-CM demonstrated reduced survival compared with patients with HF (p<0.001). Compared with patients with HF, clinical identification of carpal tunnel syndrome, spinal stenosis, and atrioventricular and left bundle branch block can facilitate earlier diagnosis of ATTR-CM.Conclusions This study provides the first nationwide estimates of ATTR-CM prevalence and risk factors. The results reinforce the severity of the disease and the importance of earlier diagnosis, especially for female patients, in order to allow effective treatment and prevention of disease progression.Data may be obtained from a third party and are not publicly available. All data were obtained from the Swedish National Board of Health and Welfare, the data holder of the national registers used in this study. Data can be obtained from the Swedish National Board of Health and Welfare upon approval from the Swedish Ethical Review Authority.