amyloid cardiomyopathy - piedmont · clinical trial involvement: nih, dcri, st jude medical, ......
TRANSCRIPT
Amyloid Cardiomyopathy
Catherine Marti, MD MScAdvanced Heart Failure and Transplant Cardiomyopathy
Piedmont Heart InstituteAthens, GA
Disclosures
Consulting: Novartis, Abbott, St Jude
Grant support: AHA and NIH
Clinical trial involvement: NIH, DCRI, St Jude Medical, Amgen and Abbott
Objectives
• Understand the pathophysiology of Amyloid disease and different phenotypes
• Describe the differences in AL amyloid and TTR amyloid – wildtype or inherited – as relates to clinical presentation and approach to disease management
• Outline the various therapies for AL and TTR amyloid related cardiomyopathy
“Amyloidosis is a protein-misfoldingdisorder characterized by the deposition of insoluble abnormal amyloid fibrils into tissues, leading to organ dysfunction”
Amyloid Cardiomyopathy
Classification of Amyloid by Type
Prognosis – Amyloid by Subtype
J Am Heart Assoc. 2016;5:e002877
Diagnosis
Epidemiology of AL Amyloid
Pathophysiology of Amyloid
Overlap with Multiple Myeloma
AL Amyloid Presentation
Clinical Symptoms and Signs of AL Cardiac Amyloid
Evaluation of AL Amyloid
Nuclear Imaging: Selective for TTR
Echo and Amyloidosis
Advanced Echocardiographic Techniques and Cardiac Amyloidosis
Greater Restriction of Basal Compared to Apical Movement
Classic Amyloid CMR Finding
Cardiac MRI
Screening for AL Cardiac Amyloid
How sensitive is biopsy?
Importance of Cardiac Involvement in AL Amyloidosis
Prognosis and AL Cardiac Amyloid
AL Cardiac Treatment
TTR Cardiomyopathy
Diagnosis of TTR Amyloid
Genotype-phenotype correlation in ATTR Amyloid
PYP testing
Emerging Therapies for TTR
Its complicated!
Key Points
• Cardiac amyloidosis should be suspected in any patient with HFpEF or infiltrative cardiomyopathy
• Histologic diagnosis of amyloid requires further investigation to determine the protein subunit type, because therapies vary widely
• Preferred therapies for immunoglobulin light-chain amyloidosis involves standard-dose or high-dose chemo with stem-cell rescue
• New therapies for TTR cardiomyopathy are developing all of the time – stay tuned!
Diflunisal
