**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Vincenzo Castiglione1,2
1. Fondazione Toscana Gabriele Monasterio, Pisa, Italy;
2. Health Science Interdisciplinary Center, Scuola Superiore Sant’Anna, Pisa, Italy.

**Main Body of the Abstract**
Background: Recent advances in imaging and increased disease awareness have led to a significant rise in newly diagnosed cases of cardiac amyloidosis (CA). However, comprehensive epidemiological data on the disease's true incidence are lacking.
Methods: Suspected CA cases underwent diagnostic work-up in 20 Centers in Tuscany and Umbria within the CARRY registry, from January 1st to December 31st, 2022.
Results: Out of 551 subjects enrolled, 448 had complete diagnostic data, revealing CA in 179 (40%) patients: 28 (16%) light chain CA, 114 (64%) wild-type transthyretin CA, 12 (7%) variant transthyretin CA, and 25 (13%) undetermined CA. The estimated cumulative incidence was 39.6 cases per million inhabitants per year (2022 population in Tuscany and Umbria=4,518,388, according to the Italian National Institute of Statistics). Alternative diagnoses included hypertensive heart disease (n=157, 58%), hypertrophic cardiomyopathy (n=25, 9%), moderate-to-severe valvular heart disease (n=11, 4%), or a mixed phenotype (n=72, 27%). Primary referral pathways for CA suspicion were cardiological (n=345, 77%), incidental finding (n=58, 13%), and haematology referral (n=33, 7%). Patients with confirmed CA were older and more frequently reported musculoskeletal red flags, such as carpal tunnel syndrome and spontaneous tendon rupture. Median time to diagnosis from suspicion was similar for CA and alternative diagnoses (73 [37-140] vs. 84 [36-156] days).
Conclusions: CA diagnoses are increasing globally, with an estimated cumulative incidence of 39.6 new cases per million inhabitants per year in two large Italian regions. These findings should inform policymakers in redefining CA as a rare disease, potentially influencing patient care and treatment costs.



**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Vincenzo Castiglione^1,2^
1. Fondazione Toscana Gabriele Monasterio, Pisa, Italy;
2. Health Science Interdisciplinary Center, Scuola Superiore Sant’Anna, Pisa, Italy.

**Main Body of the Abstract**
**Background:** Recent advances in imaging and increased disease awareness have led to a significant rise in newly diagnosed cases of cardiac amyloidosis (CA). However, comprehensive epidemiological data on the disease's true incidence are lacking.
**Methods**: Suspected CA cases underwent diagnostic work-up in 20 Centers in Tuscany and Umbria within the CARRY registry, from January 1st to December 31st, 2022.
**Results:** Out of 551 subjects enrolled, 448 had complete diagnostic data, revealing CA in 179 (40%) patients: 28 (16%) light chain CA, 114 (64%) wild-type transthyretin CA, 12 (7%) variant transthyretin CA, and 25 (13%) undetermined CA. The estimated cumulative incidence was 39.6 cases per million inhabitants per year (2022 population in Tuscany and Umbria=4,518,388, according to the Italian National Institute of Statistics). Alternative diagnoses included hypertensive heart disease (n=157, 58%), hypertrophic cardiomyopathy (n=25, 9%), moderate-to-severe valvular heart disease (n=11, 4%), or a mixed phenotype (n=72, 27%). Primary referral pathways for CA suspicion were cardiological (n=345, 77%), incidental finding (n=58, 13%), and haematology referral (n=33, 7%). Patients with confirmed CA were older and more frequently reported musculoskeletal red flags, such as carpal tunnel syndrome and spontaneous tendon rupture. Median time to diagnosis from suspicion was similar for CA and alternative diagnoses (73 [37-140] vs. 84 [36-156] days).
**Conclusions:** CA diagnoses are increasing globally, with an estimated cumulative incidence of 39.6 new cases per million inhabitants per year in two large Italian regions. These findings should inform policymakers in redefining CA as a rare disease, potentially influencing patient care and treatment costs.



Incidence and Clinical Characteristics of Cardiac Amyloidosis in Italy PB79 (#364)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.