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This editorial refers to ‘Mechanisms of heart failure in transthyretin vs. light chain amyloidosis’, by C. Binder et al., pp. 512–524.

Cardiac amyloidosis is a malignant disease presenting with cardiac hypertrophy and heart failure. The condition is probably severely underdiagnosed. Imaging efforts have mainly focused on establishing the diagnosis of cardiac amyloidosis. The discrimination from other aetiologies of hypertrophy can have important clinical implications. Sarcomere disease in genetic hypertrophic cardiomyopathy requires a different approach and management, and other causes of hypertrophy and heart failure may require specific treatments, highlighting the importance of correct diagnosis.

Also in subgroups of cardiac amyloidosis it is important to establish the correct aetiology. Recognizing light chain amyloidosis (AL) triggers a search for an underlying malignant process, with potential therapeutic targets, albeit with a limited effect on the cardiac disease manifestation and the severe prognosis. The genetic form of transthyretin (TTR) amyloidosis also represents a severe diagnosis and may even require highest specialized treatment including liver transplantation. For the most common subtype, the TTR wild-type amyloidosis (ATTRw), there have so far been limited therapeutic possibilities beyond traditional heart failure treatment. Therefore, cardiac imaging has primarily addressed differential diagnoses and optimization of heart failure treatment, and a few studies have reported the value in estimating myocardial amyloid burden,1 and strict prognostication.2 Importantly, recent reports indicate a promising therapy of ATTRw.3 This perspective urges for further knowledge of amyloid disease progression and markers of adverse outcome.

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