**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.**
Mazen Hanna, MD, Cleveland Clinic, Cleveland, OH

**Main Body of the Abstract**
Introduction: There has been significant increase in the rate of diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) due to improved disease recognition and availability of therapy. While both variant and wild-type ATTR-CM prevalence increased overtime,  the evolution of their comparative outcomes remains to be defined.
Methods: We enrolled patients with ATTR-CM who were diagnosed between 2001 and 2021 at 5 amyloidosis centers in the US. Patients were analyzed according to their genotype status. Primary outcome was all-cause mortality. To compare patients outcomes over time, we classified patients according to the era of diagnosis: pre-2010, 2010-2013, 2014-2017, and 2018-2021.
Results: We enrolled 1,168 patients (14.1% women, 21.8% variant ATTR-CM and 53.4% received tafamidis at any point during follow-up). While numerically the number of patients with variant ATTR-CM increased overtime, the proportion of patients decreased: 48.8%, 43%, 19.1%, 16.4% in pre-2010, 2010-2013, 2014-2017, 2018-2021, respectively. In patients with wild type ATTR-CM, there was no pattern of change of survival over the 4 eras (p=0.2), while there was significant improvement in survival in patients with variant ATTR-CM, p=0.00061. Data analysis is ongoing and will present granular details and provide multivariable models at time of presentation.
Conclusion: Over 20 years, the proportion of patients diagnosed with variant ATTR-CM is declining. Survival significantly improved in the variant but not wild-type ATTR-CM patients. All-cause mortality burden remains substantial in patients with ATTR-CM



**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.**
Mazen Hanna, MD, Cleveland Clinic, Cleveland, OH

**Main Body of the Abstract**
**Introduction:** There has been significant increase in the rate of diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) due to improved disease recognition and availability of therapy. While both variant and wild-type ATTR-CM prevalence increased overtime,  the evolution of their comparative outcomes remains to be defined.
**Methods:** We enrolled patients with ATTR-CM who were diagnosed between 2001 and 2021 at 5 amyloidosis centers in the US. Patients were analyzed according to their genotype status. Primary outcome was all-cause mortality. To compare patients outcomes over time, we classified patients according to the era of diagnosis: pre-2010, 2010-2013, 2014-2017, and 2018-2021.
**Results:** We enrolled 1,168 patients (14.1% women, 21.8% variant ATTR-CM and 53.4% received tafamidis at any point during follow-up). While numerically the number of patients with variant ATTR-CM increased overtime, the proportion of patients decreased: 48.8%, 43%, 19.1%, 16.4% in pre-2010, 2010-2013, 2014-2017, 2018-2021, respectively. In patients with wild type ATTR-CM, there was no pattern of change of survival over the 4 eras (p=0.2), while there was significant improvement in survival in patients with variant ATTR-CM, p=0.00061. Data analysis is ongoing and will present granular details and provide multivariable models at time of presentation.
**Conclusion**: Over 20 years, the proportion of patients diagnosed with variant ATTR-CM is declining. Survival significantly improved in the variant but not wild-type ATTR-CM patients. All-cause mortality burden remains substantial in patients with ATTR-CM



Survival in Wild-Type and Variant Transthyretin Amyloid Cardiomyopathy Over a 20-Year Period PC81 (#566)

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.