093 Changing Picture of ATTR Wild Type Cardiomyopathy in Australia – Queensland Amyloidosis Centre Experience

Abstract

Queensland Amyloidosis Centre was established over 10 years ago to deliver the multidisciplinary specialist services to patients with AL and ATTR amyloidosis. From 2017 we have observed a significant increase in referrals of patients with ATTRwt cardiomyopathy which coincided with the broader implementation of new diagnostic tool (⁹⁹Tc bone scintigraphy). We present an epidemiological and clinical data on our cohort with confirmed wild type TTR cardiac deposition. We analysed the group according to the year of referral (before vs after 2017). Our 103 patients are predominantly male (98 male, 5 female) with median age of 78 years (range, 57- 91). On presentation 42% were in Gilmore stage 1 and 40% had reduced LV ejection fraction (LVEF<50%). The average LV wall thickness (IVS) was 1.7cm and global longitudinal strain GLS (-12.8%). The observed 2 years mortality was 23%. The introduction of ⁹⁹Tc-DPD bone scintigraphy resulted in a shift to a non-biopsy pathway for diagnosis of ATTR cardiomyopathy. Positive ⁹⁹Tc-DPD scan (Perugini grade 2 and 3) obviated the need for tissue (cardiac) biopsy in majority of cases. We observed a reduction in natriuretic peptides levels, LV wall thickness and improvement in LV ejection fraction, GLS and clinical stage in patients referred after 2017. We also noted a change in 2-year survival: 69% (before 2017) vs 81% (after 2017). The early recognition of the disease due to an increased clinicians’ awareness and the advances in diagnostic workup are changing the clinical landscape of ATTR cardiomyopathy in Australia.Table 1Characteristics of 103 patients with ATTRwt cardiomyopathy ∼ year of referral, mode of dgn: ⁹⁹Tc- DPD bone scintigraphy (Dgn⁹⁹Tc) vs biopsy (DgnTiss), interventricular wall thickness (IVS), LV ejection fraction (EF), Global Longitudinal Strain (GLS), average BNP, NT-proBNP levels, %Gilmore Stage 1 (NT- proBNP<3000ng/l or BNP<400ng/l and eGFR>45ml/min) and survival at 2 years.Year/NoPatientsDgn⁹⁹TcDgnTissIVS cmEF%GLS%BNP ng/lNT-pBNP ng/lGilmoreStage 1Survival2 yearPre 173627%1173%251.849-10580732440%69%Post 176770%4930%181.656-16463292643%81% Open table in a new tab