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Concurrent Use of Tafamidis and Mavacamten for Transthyretin Cardiac Amyloidosis With Severe Outflow Tract

Ali Asghar Kassamali1, Joban Vaishnav2, Matthew J Czarny2

  • 1Johns Hopkins University, Krieger School for Arts and Science, Baltimore, Maryland, USA.

JACC. Case Reports
|August 8, 2025
PubMed

Insights

Cardiac amyloidosis (CA) can mimic hypertrophic cardiomyopathy, presenting diagnostic challenges. Concurrent treatment with mavacamten and tafamidis effectively manages obstructive CA, improving symptoms and slowing progression.

Area of Science:

  • Cardiology
  • Cardiovascular Medicine
  • Amyloidosis Research

Background:

  • Cardiac amyloidosis (CA) and hypertrophic cardiomyopathy share overlapping clinical features.
  • Diagnostic and management challenges arise from these similarities.

Observation:

  • A case of a 79-year-old man with exertional dyspnea and presyncope is presented.
  • The patient was diagnosed with transthyretin CA with left ventricular outflow tract (LVOT) obstruction.

Findings:

  • Hemodynamically significant LVOT obstruction is a rare but critical manifestation of CA.
  • Overlapping phenotypes between hypertrophic cardiomyopathy and CA can lead to diagnostic ambiguity.

Implications:

  • Endomyocardial biopsy may be necessary for definitive diagnosis when noninvasive tests are inconclusive.
  • Combined therapy with mavacamten and tafamidis offers a safe and effective treatment strategy for obstructive transthyretin CA.
  • This approach can alleviate symptoms and impede disease advancement.
Abstract

Keywords:
echocardiographyhypertrophic cardiomyopathyleft ventricular hypertrophyleft ventricular outflow tract obstructionmavacamtentafamidistransthyretin cardiac amyloidosis

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