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Critical Comparison of Documents From Scientific Societies on Cardiac Amyloidosis: JACC State-of-the-Art Review
Claudio Rapezzi1, Alberto Aimo2, Matteo Serenelli3
1Cardiologic Centre, University of Ferrara, Cona, Italy; Maria Cecilia Hospital, GVM Care & Research, Cotignola, Italy.
Insights
Five scientific societies released cardiac amyloidosis (CA) guidelines, emphasizing noninvasive diagnosis and new therapies. However, inconsistencies in diagnosis, risk stratification, and treatment highlight knowledge gaps for managing this condition.
Area of Science:
- Cardiology
- Medical Guidelines
Background:
- Cardiac amyloidosis (CA) is an emerging clinical challenge.
- Recent guidelines from 5 scientific societies aim to standardize CA diagnosis and management.
- These documents address diagnostic algorithms, noninvasive diagnostic methods, and novel therapies.
Purpose of the Study:
- To compare and contrast the recommendations from 5 national/international scientific society documents on cardiac amyloidosis.
- To identify similarities and differences in diagnostic criteria, risk stratification, and treatment strategies.
- To highlight knowledge gaps and unmet needs in CA management.
Main Methods:
- Comparative analysis of 5 scientific society documents on cardiac amyloidosis.
- Focus on diagnostic algorithms, noninvasive diagnostic approaches (bone scintigraphy, monoclonal protein exclusion), and treatment indications.
- Evaluation of recommendations for symptomatic CA, with or without peripheral neuropathy.
Main Results:
- All documents recommend diagnostic algorithms and emphasize noninvasive diagnosis.
- Novel disease-modifying therapies are indicated for symptomatic CA.
- Significant divergence exists among the documents regarding specific diagnostic details, risk stratification, and treatment protocols.
Conclusions:
- While guidelines offer valuable direction, inconsistencies create "gray zones" in CA management.
- Further research is needed to address discrepancies in diagnosis, risk stratification, and treatment.
- Standardization of CA care requires further investigation and consensus building.
Abstract:
Over the last year, 5 national or international scientific societies have issued documents regarding cardiac amyloidosis (CA) to highlight the emerging clinical science, raise awareness, and facilitate diagnosis and management of CA. These documents provide useful guidance for clinicians managing patients with CA, and all include: 1) an algorithm to establish a diagnosis; 2) an emphasis on noninvasive diagnosis with the combined use of bone scintigraphy and the exclusion of a monoclonal protein; and 3) indications for novel disease-modifying therapies for symptomatic CA, either with or without peripheral neuropathy. Nonetheless, the documents diverge on specific details of diagnosis, risk stratification, and treatment. Highlighting the similarities and differences of the documents by the 5 scientific societies with respect to diagnosis, risk stratification, and treatment offers useful insight into the knowledge gaps and unmet needs in the management of CA. An analysis of these documents, therefore, highlights "gray zones" requiring further investigation.
