Panelists discuss how monitoring clinical indicators, cardiac imaging, biomarkers, and functional assessments—such as NT-proBNP, troponin, and the 6-minute walk test—helps detect progression in transthyretin amyloid cardiomyopathy (ATTR-CM), allowing for early intervention and optimal disease management.
Summary for Physicians: Prognostic Indicators and Detection of Progression in ATTR-CM
In ATTR-CM (transthyretin amyloid cardiomyopathy), identifying and monitoring progression is crucial for optimal patient management. Several prognostic indicators and methods for detecting progression include:
Clinical Indicators:
Worsening heart failure symptoms: Increasing shortness of breath, fatigue, and edema suggest cardiac deterioration.
Arrhythmias: New-onset atrial fibrillation, sustained ventricular arrhythmias, or worsening arrhythmias signal disease progression.
Decline in functional status: A reduction in exercise tolerance or worsening ability to perform daily activities.
Cardiac Imaging:
Echocardiography: Progression can be indicated by worsening left ventricular ejection fraction, increased wall thickness, or diastolic dysfunction.
Cardiac MRI: Increased gadolinium retention (suggesting amyloid deposition) or worsening myocardial edema may indicate progression.
Bone scintigraphy: In patients with bone tracer uptake, an increase in uptake may reflect more extensive amyloid deposition.
Biomarkers:
Elevated NT-proBNP: An increase in NT-proBNP levels indicates worsening heart failure and may suggest progression.
Elevated troponin: Continuous or rising levels of cardiac troponin reflect myocardial injury and may signal disease progression.
Free light chains and TTR levels: Changes in these biomarkers may indicate disease progression or response to treatment.
Functional assessments:
Six-minute walk test: A decline in the distance walked may reflect worsening functional status.
Quality-of-life measures: Tools assessing functional capacity and patient-reported symptoms, such as the Kansas City Cardiomyopathy Questionnaire, can provide insight into disease progression.
Other factors:
TTR stabilization therapies: Lack of response or disease stabilization despite treatment may signal progression.
Systemic involvement: Worsening symptoms of other organ involvement (eg, neuropathy, carpal tunnel syndrome) may also be indicative of disease progression.
In summary, progression in ATTR-CM is defined by a combination of clinical, imaging, biomarker, and functional assessments. Monitoring these factors regularly helps physicians detect progression early, enabling timely interventions to manage the disease effectively.