ATTR Amyloidosis: Current and Emerging Management Strategies: JACC: CardioOncology State-of-the-Art Review

Jan M. Griffin, Julie L. Rosenthal, Justin L. Grodin, Mathew S. Maurer, Martha Grogan, Richard K. Cheng

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

Transthyretin cardiac amyloidosis (ATTR-CA) is increasingly diagnosed owing to the emergence of noninvasive imaging and improved awareness. Clinical penetrance of pathogenic alleles is not complete and therefore there is a large cohort of asymptomatic transthyretin variant carriers. Screening strategies, monitoring, and treatment of subclinical ATTR-CA requires further study. Perhaps the most important translational triumph has been the development of effective therapies that have emerged from a biological understanding of ATTR-CA pathophysiology. These include recently proven strategies of transthyretin protein stabilization and silencing of transthyretin production. Data on neurohormonal blockade in ATTR-CA are limited, with the primary focus of medical therapy on judicious fluid management. Atrial fibrillation is common and requires anticoagulation owing to the propensity for thrombus formation. Although conduction disease and ventricular arrhythmias frequently occur, little is known regarding optimal management. Finally, aortic stenosis and ATTR-CA frequently coexist, and transcatheter valve replacement is the preferred treatment approach.

Original languageEnglish (US)
Pages (from-to)488-505
Number of pages18
JournalJACC: CardioOncology
Volume3
Issue number4
DOIs
StatePublished - Oct 2021

Keywords

  • amyloidosis
  • cardiomyopathy
  • heart failure

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Oncology

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