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Peer-reviewedCC BY-NC-ND 4.0JACC Case Reports

Extremely Severe Aortic Stenosis Mimicking Cardiac Amyloidosis on Multimodality Imaging

  • Yu Young Kim1
  • Kyoungjune Pak2,3
  • Ji Won Lee4
  • Jung Hyun Choi5,6
6 affiliations
  1. 1Department of Internal Medicine, Pusan National University Hospital, Busan, Republic of Korea
  2. 2Department of Nuclear Medicine, Pusan National University Hospital, Busan, Republic of Korea
  3. 3School of Medicine, Pusan National University, Busan, Republic of Korea
  4. 4Department of Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Busan, South Korea
  5. 5Division of Cardiology, Department of Internal Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Republic of Korea
  6. 6Department of Internal Medicine, Pusan National University School of Medicine, Busan, Republic of Korea
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Abstract

Background: Cardiac amyloidosis is increasingly recognized in older patients with severe aortic stenosis, yet advanced pressure-overload remodeling can produce overlapping imaging features that confound noninvasive diagnosis.

Case Summary: A 70-year-old woman presented with progressive exertional dyspnea and markedly elevated cardiac biomarkers. Transthoracic echocardiography revealed extremely severe calcific aortic stenosis, increased left ventricular wall thickness, and severely reduced global longitudinal strain. Cardiac magnetic resonance imaging demonstrated elevated native T1 and extracellular volume with late gadolinium enhancement, and cardiac amyloid radionuclide imaging showed Perugini grade 1 uptake. Despite clinical suspicion of cardiac amyloidosis, noninvasive findings remained inconclusive. The patient underwent surgical aortic valve replacement with intraoperative myocardial biopsy, which demonstrated cardiomyocyte hypertrophy and interstitial fibrosis without amyloid deposition, consistent with advanced pressure-overload remodeling rather than infiltrative disease.

Discussion: Extremely severe aortic stenosis can mimic cardiac amyloidosis across multiple imaging modalities. Histologic confirmation remains essential when noninvasive findings are discordant or inconclusive.

Take-Home Messages: Severe aortic stenosis may closely mimic cardiac amyloidosis on multimodality imaging. Histological confirmation can be decisive in resolving diagnostic uncertainty.

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Cite

BibTeXRIS
BibTeX
@article{kim2026extremely,
  title = {{Extremely Severe Aortic Stenosis Mimicking Cardiac Amyloidosis on Multimodality Imaging}},
  author = {Yu Young Kim and Kyoungjune Pak and Ji Won Lee and Jung Hyun Choi},
  journal = {JACC Case Reports},
  year = {2026},
  month = oct,
  doi = {10.1016/j.jaccas.2026.110353},
  url = {https://doi.org/10.1016/j.jaccas.2026.110353},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Extremely Severe Aortic Stenosis Mimicking Cardiac Amyloidosis on Multimodality Imaging
AU  - Yu Young Kim
AU  - Kyoungjune Pak
AU  - Ji Won Lee
AU  - Jung Hyun Choi
PY  - 2026
DA  - 2026/10/01
JO  - JACC Case Reports
DO  - 10.1016/j.jaccas.2026.110353
UR  - https://doi.org/10.1016/j.jaccas.2026.110353
AB  - Background: Cardiac amyloidosis is increasingly recognized in older patients with severe aortic stenosis, yet advanced pressure-overload remodeling can produce overlapping imaging features that confound noninvasive diagnosis.  Case Summary: A 70-year-old woman presented with progressive exertional dyspnea and markedly elevated cardiac biomarkers. Transthoracic echocardiography revealed extremely severe calcific aortic stenosis, increased left ventricular wall thickness, and severely reduced global longitudinal strain. Cardiac magnetic resonance imaging demonstrated elevated native T1 and extracellular volume with late gadolinium enhancement, and cardiac amyloid radionuclide imaging showed Perugini grade 1 uptake. Despite clinical suspicion of cardiac amyloidosis, noninvasive findings remained inconclusive. The patient underwent surgical aortic valve replacement with intraoperative myocardial biopsy, which demonstrated cardiomyocyte hypertrophy and interstitial fibrosis without amyloid deposition, consistent with advanced pressure-overload remodeling rather than infiltrative disease.  Discussion: Extremely severe aortic stenosis can mimic cardiac amyloidosis across multiple imaging modalities. Histologic confirmation remains essential when noninvasive findings are discordant or inconclusive.  Take-Home Messages: Severe aortic stenosis may closely mimic cardiac amyloidosis on multimodality imaging. Histological confirmation can be decisive in resolving diagnostic uncertainty.
SN  - 2666-0849
ER  -