Unmasking the Prevalence of AC in an Unselected Echocardiographic Population

A National Survey of Prevalence and Accuracy of Echocardiographic Red Flags of Amyloid Cardiomyopathy in Consecutive Patients Undergoing Routine Echocardiography.

This study will investigate the prevalence of echocardiographic red-flags of amyloid cardiomyopathy (AC) in patients undergoing clinically-indicated echocardiography (observational phase) and the prevalence of AC among AC-suggestive echocardiograms (interventional phase).

Prospective multicenter Italian survey consisting of two phases: observational (echocardiographic screening for amyloid cardiomyopathy among patients ≥ 55 years undergoing clinically-indicated echocardiography) and interventional (specific clinical and instrumental work-up to detect the prevalence of amyloid cardiomyopathy among amyloid cardiomyopathy-suggestive echocardiograms).

Inclusion Criteria:

All the following:

• Interventricular septum thickness ≥ 13 mm in men and ≥ 12 mm in women;
• Left ventricular ejection fraction ≥ 50%;
• Indexed end-diastolic left ventricular volume ≤ 85 mL/m2.

AND

At least one of the following criteria:

• "Granular sparkling" appearance of the myocardium defined granular texture with uniform increased brightness of echo-reflections;
• Pericardial effusion regardless of severity;
• Increased interatrial septum thickness (> 5 mm);
• Restrictive filling pattern (Dec. Time E wave <120 ms or Dec. Time E wave ≤150 ms e E/A ratio ≥2) or increased ventricular filling pressures (E/E'15);
• Speckle tracking derived global longitudinal strain with apical sparing pattern;
• Increased thickness (> 5 mm) of mitral and tricuspid valve leaflets.

Exclusion Criteria:

• Patients aged < 55 years or > 100 years
• Echocardiography performed due to known or suspected amyloid cardiomyopathy;
• Echocardiography performed due to known hypertrophic cardiomyopathy or phenocopies;
• Refuse to sign the informed consent to the study