amyloid cardiomyopathy - piedmont · clinical trial involvement: nih, dcri, st jude medical, ......

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!

Questions?

Catherine.marti@piedmont.org

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