When amyloid chooses sides: septal affection pattern as an atypical presentation of transthyretin amyloid cardiomyopathy.
Lea A Maurer, Annina A Studer Bruengger, Muriel S Wiedemann Buser et al.
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Case in brief
A 73-year-old man with wild-type transthyretin amyloid cardiomyopathy had an atypical pattern of cardiac involvement on multimodality imaging. Technetium-99m DPD scintigraphy showed uptake almost exclusively in the ventricular septum. Cardiovascular magnetic resonance likewise demonstrated predominantly septal diffuse late gadolinium enhancement and elevated extracellular volume, while echocardiographic strain showed markedly impaired longitudinal strain in septal LV segments. The findings illustrate that ATTR-CM can have a markedly asymmetric distribution rather than the classically described pattern of more symmetrical basal and midventricular involvement.
What made this case unusual
Amyloid involvement was concentrated almost exclusively in the ventricular septum across scintigraphy, CMR, and echocardiographic strain—an atypical distribution for ATTR-CM.
Diagnostic / clinical pearl
A septum-predominant pattern on cardiac imaging does not exclude ATTR-CM; interpret scintigraphy, CMR, and strain findings together.