ASSUMER on PYP Scan for Cardiac Amyloid Typing and Origin: Case-Specific Corroborating Clinical Background and Genetic Analysis

Journal of Biomedical Research & Environmental Sciences(2023)

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摘要
Suspicion for Cardiac Amyloidosis (CA) led a 75-year-old male patient with fatigue, dyspnea, and palpitations to be subjected to scintigraphic myocardial investigation with technetium-99m-pyrophosphate (99mTc-PYP, or PYP scan) in order to distinguish transthyretin-related CA (ATTR) from the light-chain Amyloidosis (AL) form. A characteristic anamnesis of bilateral carpal tunnel syndrome, vertebral canal stenosis, and senile cataract, as well as laboratory, Electrocardiographic (ECG), and echocardiographic exploration was compatible with a positive for ATTR PYP scan. Specific features on PYP scan, namely Apical Sparing, more marked Septal Uptake and an associated Equivalent cardiac retention, particularly heart-to-contralateral (H/CL) Ratio from 1 h to 3 h pi (“ASSUMER”) were coupled with a negative genetic analysis, verifying wild-type ATTR. The herein introduced “ASSUMER” on PYP scan could be a hallmark not only of the amyloid fibril subtype but also origin.
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关键词
cardiac amyloid typing,pyp scan,genetic analysis,case-specific
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