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Hot Hearts on Bone Scintigraphy Are Not All Amyloidosis: Hyperoxaluria-Associated Cardiomyopathy
Ya-Wen Wang1, Yan-Ting Xie2, Xiao-Xin Sun
1From the Department of Nuclear Medicine, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing.
Insights
Primary hyperoxaluria can cause a false positive 99m Tc-PYP scan in patients with infiltrative cardiomyopathy. This highlights the importance of considering non-amyloid causes of cardiac uptake in pyrophosphate imaging.
Area of Science:
- Nephrology
- Cardiology
- Nuclear Medicine
Background:
- End-stage renal disease (ESRD) patients may develop infiltrative cardiomyopathy.
- 99m Tc-pyrophosphate (99m Tc-PYP) scintigraphy is used to detect cardiac amyloidosis.
Observation:
- A 54-year-old woman with ESRD presented with infiltrative cardiomyopathy.
- Echocardiography revealed infiltrative cardiomyopathy.
- 99m Tc-PYP scintigraphy showed significant myocardial uptake.
Findings:
- Genetic testing identified an AGXT gene mutation, confirming primary hyperoxaluria.
- Myocardial uptake on 99m Tc-PYP scan was attributed to oxalate deposition in the heart.
- This case demonstrates a false positive 99m Tc-PYP scan due to hyperoxaluria-associated cardiomyopathy.
Implications:
- Primary hyperoxaluria should be considered in the differential diagnosis of myocardial uptake on 99m Tc-PYP scans.
- This finding expands the understanding of conditions mimicking cardiac amyloidosis.
- Awareness of non-amyloid etiologies can improve diagnostic accuracy in cardiomyopathy evaluations.
Abstract:
A 54-year-old woman with a history of end-stage renal disease was found to have infiltrative cardiomyopathy by echocardiography. 99m Tc-pyrophosphate ( 99m Tc-PYP) scintigraphy was positive with a remarkable myocardial uptake. Gene test found a mutation of AGXT , confirming a final diagnosis of primary hyperoxaluria. Radiotracer uptake was due to high myocardial oxalate deposition. This case illustrates false positivity of the 99m Tc-PYP scan caused by hyperoxaluria-associated cardiomyopathy, which raises awareness for other conditions apart from amyloid cardiomyopathy.
