Beyond the Valve and into the Muscle: A Review of Coexisting Aortic Stenosis and Transthyretin Cardiac Amyloidosis

ABSTRACT

The concurrence of transthyretic cardiac amyloidosis (ATTR-CA) and severe aortic stenosis (AS) is being increasingly identified, however the long-term trajectory of these patients remains unclear. In this comprehensive review, we discuss what is known about the prevalence of dual diagnosis, the impact of new imaging modalities on diagnosis of ATTR-CA, and outcomes for patients with concomitant disease. We discuss emerging medical therapies for ATTR-CA, which may be considered in appropriate patients with ATTR-CA and severe AS.

KEYWORDS:

• Transthyretin cardiac amyloidosis
• aortic stenosis
• cardiac amyloidosis

Disclosure statement

Dr. Hahn reports speaker fees from Boston Scientific Corporation and Baylis Medical; consulting for Abbott Structural, Edwards Lifesciences, Medtronic, Navigate, Philips Healthcare and Siemens Healthcare; non-financial support from 3mensio; and is the Chief Scientific Officer for the Echocardiography Core Laboratory at the Cardiovascular Research Foundation for multiple industry-sponsored trials, for which she receives no direct industry compensation.

At the time of the majority of this writing and manuscript development, Dr. Castaño was Assistant Professor of Medicine and Co-Director of the Columbia University Center for Cardiac Amyloidosis; Dr. Castaño is now a full-time employee of Pfizer Inc.

Dr. Maurer reports grant support from NIH R01HL139671-01, R21AG058348 and K24AG036778, consulting income from Pfizer, GSK, EIdos, Prothena, Akcea and Alnylam, and institution received clinical trial funding from Pfizer, Prothena, Eidos and Alnylam.