Amyloidosis of the heart: state of the art

• Amyloid vs Amyloidosis
• ATTR Amyloidosis
• Transthyretin Amyloidosis (Wild-type ATTR and Hereditary ATTR)
• Electrocardiogram: what to look for
• Transthyretin Amyloidosis: non invasive diagnosis
• Why ATTR amyloidosis

• The early study in AA and AL amyloidosis established the treatment paradigm for all types of amyloidosis: deep reduction of amyloid precursor and prognosis
• Reducing amyloid production with stabilisers was unequivocally associated with better outcome
• ATTR amyloidosis became the prototype clinical indication for the gene silencers programmes
• Expanding options: gene editing (Nex-z)
• Accelerating amyloid removal to ‘complement’ slowing production remains the ‘holy grail’
• Change in disease understanding: from a disease of misfolding to a disease of clearance

Professor Marianna Fontana is professor of cardiology and honorary consultant cardiologist at the Royal Free Hospital, University College London, where she is Director of the UCL Cardiac MRI unit, deputy head of center for Amyloidosis and deputy clinical lead for the National Amyloidosis Center. Her major clinical and research interests are in the delivery of efficient and effective care for patients with amyloidosis, with a particular focus on new technologies: imaging and drugs. She lectures widely in the UK and internationally on amyloidosis and organises many multi-professional educational events.