**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.**
Massimo Russo Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

**Main Body of the Abstract**
ATTRv amyloidosis due to p.Glu109Gln mutation is characterised by onset in the 5th – 6th decade, prevalent distal paraesthesias/CTS as presenting symptoms and early heart dysfunction. Heart failure and sudden death are the major cause of mortality followed by dysautonomia and cachexia. Prior to the availability of patisiran and inotersen we reported a life expectancy of 7.6±3.7 (3–13) years in a cohort of 40 patients.  The objective of this single-center real world study is the description of cardiac function in a cohort of patients with p.Glu109Gln mutation in whom patisiran therapy for polyneuropathy was started.
At time 0, six out of ten patients presented parameters consistent with a picture of cardiac hypertrophy with maintained ejection fraction with a mixed phenotype and elevated troponin T and NT pro BNP values. Four out of ten patients presented normal cardiac function parameters with only a neuropathic phenotype and normal NTproBNP and Troponin T values.
During follow-up of 27.8 months (6-54), 8 patients showed stability of echocardiographic and serological parameters ​​with absence of cardiac symptoms. One patient had a reduction in troponin values despite ultrasound parameters, NT-pro BNP remain substantially unchanged, and another one experienced a reduction of the ejection fraction with the appearance of heart failure symptoms.
In this study, the use of patisiran allowed substantial stability of the cardiological picture in the majority of patients with amyloidosis associated with the p.Glu109Gln variant. A greater number of patients and prolonged follow-up will be necessary for greater robustness of these findings.



Can patisiran stop the progression of cardiac amyloidosis in patients with ATTRE109Q amyloidosis? Description of a monocentric experience. PC76 (#487)

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.