Expert consensus on the monitoring of transthyretin amyloid cardiomyopathy

Pablo Garcia-Pavia, Frank Bengel, Dulce Brito, Thibaud Damy, Franz Duca, Sharmila Dorbala, Jose Nativi-Nicolau, Laura Obici, Claudio Rapezzi, Yoshiki Sekijima, Perry M. Elliott

Research output: Contribution to journalReview articlepeer-review

Abstract

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a life-threatening condition with a heterogeneous clinical presentation. The recent availability of treatment for ATTR-CM has stimulated increased awareness of the disease and patient identification. Stratification of patients with ATTR-CM is critical for optimal management and treatment; however, monitoring disease progression is challenging and currently lacks best-practice guidance. In this report, experts with experience in treating amyloidosis and ATTR-CM developed consensus recommendations for monitoring the course of patients with ATTR-CM and proposed meaningful thresholds and frequency for specific parameters. A set of 11 measurable features across three separate domains were evaluated: (i) clinical and functional endpoints, (ii) biomarkers and laboratory markers, and (iii) imaging and electrocardiographic parameters. Experts recommended that one marker from each of the three domains provides the minimum requirements for assessing disease progression. Assessment of cardiac disease status should be part of a multiparametric evaluation in which progression, stability or improvement of other involved systems in transthyretin amyloidosis should also be considered. Additional data from placebo arms of clinical trials and future studies assessing ATTR-CM will help to elucidate, refine and define these and other measurements.

Original languageEnglish
JournalEuropean Journal of Heart Failure
DOIs
Publication statusAccepted/In press - 2021

Keywords

  • Amyloidosis
  • Cardiac imaging
  • Heart failure
  • Laboratory markers
  • Monitoring tools
  • Transthyretin amyloid cardiomyopathy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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