**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.**
Larissa Ventura Ribeiro Bruscky. Instituto Dante Pazzanese de Cardiologia, São Paulo-SP. Brazil.

**Main Body of the Abstract**
Introduction: Amyloidosis are protein-misfolding disorders associated with deposition of amyloid proteins in multiple organs and progressive organ dysfunction. Familial transthyretin amyloidosis (ATTRm) usually presents itself as a progressive polyneuropathy, but also as cardiomyopathy, nephropathy and vitreous opacities. Single features rarely remain isolated. We describe a patient with ATTRm who had sustained ventricular tachycardia as an isolated symptom​.
CASE REPORT: A 61-year-old Brazilian man, with medical history of hypertension, Portuguese parents, complaining of chest pain followed by palpitations one hour before admission to the emergency room. Intense sweating and high heart rate (196 beats/min) were observed. ECG revealed sustained ventricular tachycardia. He was successfully submitted to electrical cardioversion and a cardioverter defibrillator was implanted. ​ ​Biological markers for myocardial necrosis were high but a coronary angiogram revealed no significant contribution. Echocardiogram showed ejection fraction of 66%, diffuse hypertrophy (septum 19mm and posterior wall 15mm), moderate atrial enlargement and relative apical sparing pattern. Cardiac magnetic resonance showed diffuse late gadolinium enhancement of non-ischemic pattern, circumferential, and myocardial edema, suggesting amyloidotic cardiomyopathy. Molecular analysis of TTR gene evidenced a p.Val50Met mutation.​
CONCLUSION: TTRm as cause of Sustained Ventricular Tachycardia should be considered.​Val50Met is a pathogenic mutation characterized, mainly, by being clinically expressed by polyneuropathy (PAF). When cardiomyopathy is present, usually heart failure is the main clinical expression. In this case, ventricular arryhthmia was the only feature.  This case highlights the risk of sudden cardiac death in ATTRm.



Sustained Ventricular Tachycardia as an isolated feature of transthyretin amyloidotic cardiomyopathy - Val50Met PB43 (#476)

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.