A more standardized approach to monitoring the progression of transthyretin amyloid cardiomyopathy (ATTR-CM) is now available, offering clinicians a framework to better assess how the disease evolves in patients. An international panel of experts recently presented updated criteria, published in
The updated criteria build upon previous guidance from 2021, recognizing the need for a more comprehensive and accurate way to track disease progression. The aim is to provide a practical, multidimensional framework suitable for routine clinical practice and to standardize follow-up procedures across different medical centers and research studies.
ATTR-CM is a condition where abnormal deposits of the protein transthyretin (TTR) build up in the heart, leading to stiffening of the heart muscle and ultimately heart failure. Monitoring disease progression is crucial for understanding how effectively treatments are working and for making informed decisions about patient care.
Six Key Parameters for Monitoring
The expert panel identified six specific parameters that should be monitored over a 12-month period to assess disease progression. These include:
- Heart failure-related hospitalization: This identifies instances where the condition worsens to the point of requiring emergency or inpatient care.
- Outpatient diuretic intensification: Tracking increases in diuretic medication dosage indicates worsening fluid retention and heart failure symptoms.
- N-terminal pro-B-type natriuretic peptide (NT-proBNP): This biomarker reflects cardiac stress and the severity of heart failure.
- Estimated glomerular filtration rate (eGFR): This measures kidney function, which can be affected by amyloid deposits.
- Six-minute walk test: This assesses a patient’s functional capacity and exercise tolerance.
- Quality of life measures: Tools like the Kansas City Cardiomyopathy Questionnaire or the New York Heart Association (NYHA) functional class provide insight into how the disease impacts a patient’s daily life.
Defining Disease Progression
According to the new criteria, disease progression should be considered when at least two of these six parameters meet predefined thresholds over a 12-month period. Importantly, clinicians are advised to compare results with those from the previous 12 months, rather than comparing to baseline measurements. This approach helps to minimize the impact of random fluctuations and provides a more accurate picture of true disease progression.
“The proposed approach emphasizes that disease progression should be determined by changes across several parameters rather than a single measure to ensure accurate detection of clinically relevant worsening,” the authors wrote.
Limitations and Future Research
While the updated criteria represent a significant step forward in standardizing ATTR-CM monitoring, the researchers acknowledge that further investigation is needed. Currently, it remains unclear whether identifying disease progression, as defined by these criteria, should automatically trigger changes in treatment plans.
The authors emphasize the need for prospective studies to determine whether applying these monitoring criteria ultimately leads to improved patient outcomes. The current framework is designed to standardize monitoring, but not to dictate specific treatment decisions at this stage.
The position statement highlights that consistent monitoring across clinics and studies may improve the ability to conduct future research and refine clinical decision-making in patients with ATTR-CM. The updated criteria aim to integrate clinical events, biomarker data, functional performance, and patient-reported outcomes for a more holistic assessment of disease progression.
As of , these updated criteria offer a valuable tool for clinicians managing patients with ATTR-CM, providing a more standardized and comprehensive approach to monitoring disease progression.
References
- García-Pavía P, Witteles R, Damy T, Maurer MS, et al. Monitoring disease progression in patients with transthyretin amyloid cardiomyopathy. JACC Heart Fail. doi:10.1016/j.jchf.2025.102766
- New proposed criteria for monitoring disease progression in ATTR-CM. ACC Journal Scan. December 10, 2025. Accessed February 4, 2026.
https://www.acc.org/Latest-in-Cardiology/Journal-Scans/2025/12/09/16/51/New-Proposed-Criteria-For-Monitoring-Disease-Progression-in-ATTR-CM
