Technologist Track Session 3: How I Do It: Patients with Cardiomyopathies-Amyloidosis

Video Transcription

Video Summary

The lecture explains cardiac amyloidosis, a protein misfolding disorder that causes starch-like (“amyloid”) deposits in the extracellular space and disrupts heart function. It is an infiltrative, restrictive cardiomyopathy that often presents with heart failure despite preserved ejection fraction, enlarged atria, increased ventricular wall thickness, and diastolic dysfunction. Two major types are discussed: light-chain (AL) amyloidosis, a hematologic and most lethal form, and transthyretin (ATTR) amyloidosis, which can be wild-type or hereditary. CMR findings include difficulty nulling the myocardium, subendocardial or diffuse late gadolinium enhancement, increased native T1, and elevated extracellular volume. Native T1 mapping is emphasized as especially valuable for early diagnosis before irreversible disease develops. Treatment includes diuretics, atrial fibrillation management, and newer targeted therapies; advanced cases may require stem cell, liver, or heart transplantation depending on the amyloid type.

Keywords

cardiac amyloidosis

restrictive cardiomyopathy

light-chain amyloidosis

transthyretin amyloidosis

cardiac MRI