Amyloid Cardiomyopathy

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  • Cardiac amyloidosis is a condition characterised by rapidly progressive heart failure and poor prognosis. The two main subtypes, immunoglobulin light chains (AL) and transthyretin (ATTR), have been investigated extensively in recent years. Cardiac imaging has advanced with the widespread use of cardiac MRI with late gadolinium enhancement imaging and newer techniques including T1 mapping to quantify amyloid burden. Nuclear imaging has developed as a highly accurate method to confirm cardiac amyloid deposits noninvasively with very high sensitivity in ATTR amyloidosis. Despite advances in imaging, cardiac biopsy remains the gold standard diagnostic test to confirm and type amyloidosis.

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    Cardiac amyloidosis is a condition characterised by rapidly progressive heart failure and poor prognosis. The two main subtypes, immunoglobulin light chains (AL) and transthyretin (ATTR), have been investigated extensively in recent years. Cardiac imaging has advanced with the widespread use of cardiac MRI with late gadolinium enhancement imaging and newer techniques including T1 mapping to quantify amyloid burden. Nuclear imaging has developed as a highly accurate method to confirm cardiac amyloid deposits noninvasively with very high sensitivity in ATTR amyloidosis. Despite advances in imaging, cardiac biopsy remains the gold standard diagnostic test to confirm and type amyloidosis.

    Amyloid cardiomyopathy diagnosis is based on either a positive cardiac biopsy demonstrating amyloid infiltration or as an increased left ventricular (LV) wall thickness (>12 mm) in the absence of arterial hypertension or other potential causes of LV hypertrophy with a positive noncardiac biopsy.

    Early diagnosis is crucial, as misdiagnosis may lead to inappropriate treatment with potentially harmful standard heart failure medications which have no evidence base in amyloidosis. Despite developments in diagnostics and therapy, the prognosis for patients with advanced cardiac involvement remains poor.

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