**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.**
Antonia S Carroll, Westmead Amyloidosis Service, Westmead Hospital, Westmead, NSW.

**Main Body of the Abstract**
Objectives
Hereditary Transthyretin amyloidosis (ATTRv) is characterised by progressive sensorimotor and autonomic neuropathy, and cardiac failure. We aim to describe the spectrum of ATTRv neuropathy in Australia.
Methods
A retrospective analysis of ATTRv patients attending Australian Amyloidosis Network clinics between 2007–2022 was performed. TTR variants, clinical features and treatments were evaluated.
Results
161 individuals were identified (62% NSW/ACT, 20% QLD, 12% VIC/TAS, 4% WA, 2% SA). Average age was 59.4 years (range 21.5–91.4). 53% were male. 37% were presymptomatic (average age 52.7 years, range 21.5–71.0). 24 genetic variants were identified, including Thr60Ala (31%), Val30Met (23%), Val122Ile (12%), Ala97Ser (6%), Glu89Gln (5%).
The diagnosis rate has increased from 2 p.a in 2011–13 to 5 p.a in 2015–17, and 12 p.a in 2020–22. The average time to diagnosis was 3.8 years (range 1–15). 13 asymptomatic individuals developed symptomatic disease over an average 2.3 years (range 0–8).
40% had neuropathic-predominant disease, 32% cardiac, 25% mixed cardiac and neuropathic, and 3% had other organ-predominant disease. Of the 130 living individuals 45% had a PND score of 0, 22% PND1,10% PND2, and 9% PND3a or above. Similarly, 53% of individuals had a FAP0, 31% FAP1, 15% FAP2 and 1% FAP3.
Of individuals with PND0–2, only 22% were on highly efficacious treatments, all via clinical trial or compassionate access scheme.
Conclusions
Our study is the first to demonstrate the spectrum of ATTRv in Australia. Affordable TTR genotyping and access to highly efficacious novel therapies remain substantial unmet needs in this population.



**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.**
Antonia S Carroll, Westmead Amyloidosis Service, Westmead Hospital, Westmead, NSW.

**Main Body of the Abstract**
**Objectives**
Hereditary Transthyretin amyloidosis (ATTRv) is characterised by progressive sensorimotor and autonomic neuropathy, and cardiac failure. We aim to describe the spectrum of ATTRv neuropathy in Australia.
**Methods**
A retrospective analysis of ATTRv patients attending Australian Amyloidosis Network clinics between 2007–2022 was performed. TTR variants, clinical features and treatments were evaluated.
**Results**
161 individuals were identified (62% NSW/ACT, 20% QLD, 12% VIC/TAS, 4% WA, 2% SA). Average age was 59.4 years (range 21.5–91.4). 53% were male. 37% were presymptomatic (average age 52.7 years, range 21.5–71.0). 24 genetic variants were identified, including Thr60Ala (31%), Val30Met (23%), Val122Ile (12%), Ala97Ser (6%), Glu89Gln (5%).
The diagnosis rate has increased from 2 p.a in 2011–13 to 5 p.a in 2015–17, and 12 p.a in 2020–22. The average time to diagnosis was 3.8 years (range 1–15). 13 asymptomatic individuals developed symptomatic disease over an average 2.3 years (range 0–8).
40% had neuropathic-predominant disease, 32% cardiac, 25% mixed cardiac and neuropathic, and 3% had other organ-predominant disease. Of the 130 living individuals 45% had a PND score of 0, 22% PND1,10% PND2, and 9% PND3a or above. Similarly, 53% of individuals had a FAP0, 31% FAP1, 15% FAP2 and 1% FAP3.
Of individuals with PND0–2, only 22% were on highly efficacious treatments, all via clinical trial or compassionate access scheme.
**Conclusions**
Our study is the first to demonstrate the spectrum of ATTRv in Australia. Affordable TTR genotyping and access to highly efficacious novel therapies remain substantial unmet needs in this population.



The spectrum of neuropathy in hereditary transthyretin amyloidosis (ATTRv) in Australia PB26 (#129)

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.