Popis: |
Introduction Transthyretin amyloidosis (ATTR) is a progressive, fatal disease caused by the formation of amyloid fibrils that misfold and accumulate in various tissue in the body, including the heart, leading to a form of heart failure called transthyretin amyloidosis cardiomyopathy (ATTR-CM). ATTR-CM consists of two subgroups: a wild-type (ATTR-wt) or an inherited mutant autosomal dominant gene (hATTR). ATTR-wt and hATTR have an estimated median survival 3.6 years and 2.5 years after diagnosis, respectively. The diagnosis of ATTR-CM is often delayed or missed, and one potential factor playing in role in this may be lack of provider awareness and knowledge of this disease. The objective of this analysis was to assess ATTR-CM knowledge of healthcare providers (HCPs) at a large academic medical center. The hypothesis of this analysis is that there is lack of foundational knowledge regarding ATTR-CM among HCPs. Methods and Results This analysis utilized a cross-sectional observational design in which HCPs were asked to voluntarily complete an electronic questionnaire. This questionnaire collected demographic characteristics, captured awareness of ATTR-CM, confidence in managing patients with ATTR-CM, and desire for additional education. Forty-six HCPs completed the survey. All respondents identified themselves as physicians, with the majority practicing in internal medicine or family medicine (43.75%). Approximately 2/3 of the HCPs had been in practice ten years or less. Most respondents reported that less than half of their current patient population have heart failure (87.5%). All respondents stated some level of awareness to ATTR-CM. However, the majority (84.79%) were not confident differentiating between hATTR and ATTR-wt, and 73.91% were not familiar with the various mutations in hereditary ATTR-CM. The survey revealed areas of knowledge gaps with majority indicating not being confident with genetic testing to determine if ATTR-CM is hereditary (73.3%), identifying the soft tissue signs and symptoms of ATTR-CM (67.39%) and differentiating between ATTR-CM and light chain amyloidosis (63.04%). The majority (67.39%) indicated a desire for further education. Conclusions Major knowledge gaps were identified in this survey indicating further education is needed to increase clinician suspicion of ATTR-CM, so patients, who may require further testing to make an accurate diagnosis, can be identified. |