Key result
Multimodal imaging including voltage mapping, cMRI, and scintigraphy successfully identifies isolated atrial amyloidosis.
Why the study?
The authors aimed to present multimodal imaging of structural and functional abnormalities detected by electrophysiological voltage mapping, cardiac magnetic resonance imaging, and 99mTc-DPD scintigraphy in isolated atrial amyloidosis.
Case Report (n=1)
Multimodal imaging, including 99mTc-DPD scintigraphy, can successfully identify isolated atrial amyloidosis in patients presenting with atrial arrhythmias and HFpEF.
Hypothesis-generating for multimodal imaging in isolated atrial amyloidosis; does not yet support practice changes.
We present not-yet-seen multimodal images of a 55-year-old female patient with isolated atrial amyloidosis (IAA) who clinically suffered from multiple atrial arrhythmias and heart failure symptoms with preserved left ventricular ejection fraction. We aim to show structural and functional abnormalities detected by electrophysiological voltage mapping, cardiac magnetic resonance imaging (MRI) [cMRI; atrial strain measurements, late gadolinium enhancement (LGE) visualization], and 99mTc-DPD scintigraphy. Bipolar voltage mapping performed during two electrophysiological procedures showed diffuse left atrial low-voltage areas (bipolar < 0.5 mV) and also a moderately diseased right atrium suspected of infiltrative cardiomyopathy. Catheter ablation did successfully treat a left atrial and two right atrial focal tachycardias. For further diagnostics, a 3T cMRI was performed, revealing a subendocardial circumferential left atrial LGE and pathological atrial strain measurements, especially during conduit and reservoir phase. Afterwards, nuclear imaging with 559 MBq of 99mTc-DPD was performed. The scan revealed amyloid infiltration of the left atrium. Neither an uptake in the ventricular myocardium nor an extra-cardiac uptake of DPD was seen. Genetic testing for transthyretin amyloidosis mutations in this patient was negative, and peripheral neuropathy was ruled out by electromyogram analysis. The synopsis of these findings reveals IAA as the most possible diagnosis and showed isolated atrial nuclear tracer uptake with 99mTc-DPD scintigraphy for the first time. Non-invasive imaging techniques might help in suggesting IAA but need further investigation.
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Schöppenthau et al. (2020) conducted a case report in Isolated atrial amyloidosis (n=1). Multimodal imaging (electrophysiological voltage mapping, cMRI, 99mTc-DPD scintigraphy) was evaluated. Multimodal imaging including electrophysiological voltage mapping, cMRI, and 99mTc-DPD scintigraphy successfully identified isolated atrial amyloidosis in a 55-year-old female patient.
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