WILD-TYPE TRANSTHYRETIN CARDIAC AMYLOIDOSIS: THE JOURNEY TO DIAGNOSIS IN THE CZECH REPUBLIC. THE RESEARCH PROJECT OF THE CZECH SOCIETY OF CARDIOLOGY

Background and aim of the study: Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) is an increasingly recognized cause of heart failure (HF) due to population ageing. The purpose of our study was to assess the journey to a correct diagnosis for ATTRwt-CA in the Czech Republic.
Methods: Between November 2022 and May 2023, data were prospectively collected from 118 ATTRwt-CA patients (86% males) followed in 4 referral centres in Czech Republic.
Results: The mean age at the time of diagnosis was 776 years. NYHA class I or II was present in 10% and 56% of patients, respectively. The diagnosis was non-invasive in 63% cases. The most frequent clinical presentation leading to the diagnosis of ATTRwt-CA were HF symptoms (62%). The history of carpal tunnel syndrome preceding the diagnosis of ATTRwt-CA was reported by 54% of patients. The median time intervals between the onset of symptoms to suspected and confirmed diagnosis were 7.5 months.
The diagnosis of ATTRwt-CA was first suspected by outpatient cardiologist in 33% of cases, followed by tertiary cardiac centre cardiologist (25%). The previous different diagnosis was present in 45% patients, most commonly hypertrophic cardiomyopathy and HF with preserved EF (40% and 26%, respectively).
Conclusions: In the Czech Republic, ATTRwt-CA is noninvasively diagnosed in majority of patients. HF symptoms represent the most common presenting clinical scenario. The history of typical cardiac or extracardiac manifestations is reported by a significant number of patients. Nevertheless, there is still a significant delay between the onset of symptoms and suspected disease, with hypertrophic cardiomyopathy and HF with preserved EF representing the most frequent misdiagnoses. Therefore, educational activities among cardiologists and other physicians are necessary to further increase the awareness of ATTRwt-CA.