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Yume Nohara-Shitama, Nobuhiro Tahara, Yoshihiro Fukumoto, The distribution of functional and molecular apical sparing in cardiac amyloidosis, European Heart Journal, 2025;, ehaf278, https://doi.org/10.1093/eurheartj/ehaf278
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Extract
An 87-year-old man was admitted to the hospital with exertional dyspnoea. Transthoracic echocardiography showed left ventricular (LV) hypertrophy with a maximum wall thickness of 18 mm and an LV ejection fraction of 57% (Panels A and B). The global longitudinal strain was decreased (13.0%) with a characteristic pattern of relative apical sparing (Panel C). Abnormal myocardial perfusion was seen in the basal and mid-LV segments (Panels D–F). Late gadolinium enhancement was observed in the basal and mid-levels of the LV, not in the apical level (Panels G–I, arrows). 99mTc-labelled hydroxy-methylene diphosphonate (HMDP) scintigraphy showed Grade 3 cardiac uptake (Panel J, arrows) with an apical sparing pattern (Panels K and L). 18F-fluorodeoxyglucose positron emission tomography demonstrated a similar LV uptake of HMDP [Xeleris (GE HealthCare, Chicago, IL)], which might reflect impaired myocardium (Panels M–O, arrows). Blood and urine testing determined the absence of monoclonal protein. No gene associated with variant transthyretin was found, leading to the diagnosis of wild-type transthyretin amyloidosis. All imaging modalities identified a relative apical sparing characteristic of cardiac amyloidosis.