**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.**
Pablo Garcia-Pavia, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain

**Main Body of the Abstract**
Background: NT-proBNP is an important biomarker that helps define disease progression in ATTR-CM. An international expert panel proposed that at an increase in NT-proBNP (>30% and >300pg/mL – Progressor Index 1) be one of the metrics signaling disease progression [Garcia-Pavia 2021]. Another recent study showed that increases in NT-proBNP >500 pg/mL (Progressor Index 2) at Month 12 after diagnosis predicted subsequent mortality in wild-type ATTR-CM [Law 2022].
The Phase 3 ATTRibute-CM study of acoramidis – a novel, potent, near-complete transthyretin (TTR) stabilizer that increases serum TTR (prealbumin) – versus placebo met its primary hierarchical efficacy endpoint with mortality, morbidity, and function components (p<0.0001). The median (IQR) baseline NT-proBNP was 2326 (1278–3910) pg/mL.
Methods: We report the rates of Progressor Indices 1 and 2 observed at 3-month intervals in ATTRibute-CM and applied Cochran-Mantel-Haenszel statistical testing for the comparison between groups.
Results: At all intervals from end of Year 1 (Months 9-12) to end of study (Months 27-30), the distribution for both indices favored acoramidis over placebo (Table).
For Index 1, a 13.5% absolute difference in the rate of progressors was observed with acoramidis at Months 9-12 (p=0.0013), increasing to reach 28.9% at Months 27-30, (p<0.0001).
For Index 2, a 14.5% absolute difference was observed with acoramidis at Months 9-12 (p=0.0005), increasing to reach 25.8% at Months 27-30, (p<0.0001).
Conclusion: ATTR-CM Progressor Indices, defined by increases in NT-proBNP, reflect disease progression and have been shown to predict subsequent mortality. Acoramidis slows disease progression as measured by significant improvement in these Progressor Indices.



**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.**
Pablo Garcia-Pavia, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain

**Main Body of the Abstract**
**Background**: NT-proBNP is an important biomarker that helps define disease progression in ATTR-CM. An international expert panel proposed that at an increase in NT-proBNP (>30% and >300pg/mL – Progressor Index 1) be one of the metrics signaling disease progression [Garcia-Pavia 2021]. Another recent study showed that increases in NT-proBNP >500 pg/mL (Progressor Index 2) at Month 12 after diagnosis predicted subsequent mortality in wild-type ATTR-CM [Law 2022].
The Phase 3 ATTRibute-CM study of acoramidis – a novel, potent, near-complete transthyretin (TTR) stabilizer that increases serum TTR (prealbumin) – versus placebo met its primary hierarchical efficacy endpoint with mortality, morbidity, and function components (p<0.0001). The median (IQR) baseline NT-proBNP was 2326 (1278–3910) pg/mL.
**Methods**: We report the rates of Progressor Indices 1 and 2 observed at 3-month intervals in ATTRibute-CM and applied Cochran-Mantel-Haenszel statistical testing for the comparison between groups.
**Results**: At all intervals from end of Year 1 (Months 9-12) to end of study (Months 27-30), the distribution for both indices favored acoramidis over placebo (Table).
For Index 1, a 13.5% absolute difference in the rate of progressors was observed with acoramidis at Months 9-12 (p=0.0013), increasing to reach 28.9% at Months 27-30, (p<0.0001).
For Index 2, a 14.5% absolute difference was observed with acoramidis at Months 9-12 (p=0.0005), increasing to reach 25.8% at Months 27-30, (p<0.0001).
**Conclusion**: ATTR-CM Progressor Indices, defined by increases in NT-proBNP, reflect disease progression and have been shown to predict subsequent mortality. Acoramidis slows disease progression as measured by significant improvement in these Progressor Indices.



Acoramidis significantly improves NT-proBNP indices that indicate ATTR-CM disease progression and predict subsequent mortality: Insights from the ATTRibute-CM Study  PC127 (#303)

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.