CONGRESS ABSTRACT

Background : ATTR-CM is caused by extracellular deposition of amyloid in the heart. Vutrisiran, an RNAi therapeutic, rapidly suppresses the hepatic production of TTR, reduces the risk of CV events and ACM among patients withATTR-CM and preserves functional capacity, health status and QoL. The present analyses evaluate the impact…

A 72-year-old male, with a medical history of hypertension, remote bilateral carpal tunnel syndrome and smoldering IgG kappa multiple myeloma (MM), was referred for evaluation of suspected cardiac amyloidosis (CA) due to mild exertional intolerance. Electrocardiogram revealed sinus rhythm, left-axis deviation, low voltage QRS. A transthoracic echocardiogram revealed reduced…

BACKGROUND : Transthyretin amyloidosis is caused by extracellular deposition of misfolded transthyretin protein and may involve the heart, leading to heart failure and arrhythmias. ATTR can be wild-type or hereditary (variant ATTR). One of the pathogenic variants is Val122Ile, prevalent in individuals of West African ancestry. Non-invasive diagnosis of…

A 64-year-old woman with a history of arterial hypertension, type 2 diabetes mellitus and end-stage chronic kidney disease on hemodialysis, who was admitted to our hospital for acute heart failure. At admission the patient was hemodynamically stable with signs of central and peripheral congestion. Electrocardiography showed sinus rhythm with…

INTRODUCTION Wild-type transthyretin cardiac amyloidosis poses a therapeutic challenge, particularly in elderly patients, requiring a balance between clinical stability, treatment cost, and management complexity. CASE In 2016, an 82-year-old male patient was admitted to the Cardiology ward after experiencing precordial pain. Coronary angiography was negative for significant coronary stenoses….

Vutrisiran is the first gene silencer to demonstrate clear clinical benefit. To date, vutrisiran has been approved for the treatment of ATTR with neuropathy following the positive results of the HELIOS-A study. The Phase 3 HELIOS-B study was developed to evaluate the drug's efficacy in patients with ATTR-CA. The…

A 48-year-old woman carrying the heterozygous Glu89Gln mutation in the TTR gene, with a family history of hereditary transthyretin amyloidosis (hATTR), underwent cardiac evaluation. She was asymptomatic from a cardiac standpoint, aside from a prior bilateral carpal tunnel syndrome, a common early manifestation of ATTR amyloidosis. Cardiac biomarkers (troponin…

Background AL amyloidosis is a rare systemic disorder related to plasma cell dyscrasias and caused by deposition of monoclonal light chains. Cardiac involvement is the main prognostic determinant. Macroglossia is an uncommon manifestation, usually described in advanced disease stages. Case Report A 61-year-old woman with obesity (BMI 39), smoking…