Ces radiol. 2024, 78(2):120-124 | DOI: 10.55095/CesRadiol2024/016

Rare detection of AL amyloidosis by 99mTc-DPD cardiac scintigraphy in a 73-year-old patient with exertional dyspnea and heart failureOriginal article

Jiří Doležal1, Petr Liška1, Miloš Baše2, Jakub Radocha3, Marie Buncová4, Miloš Kubánek5
1 Oddělení nukleární medicíny LF UK a FN, Hradec Králové
2 Kadiologická ambulance, Rychnov nad Kněžnou
3 IV. interní klinika LF UK a FN, Hradec Králové
4 Pracoviště nukleární medicíny, Institut klinické a experimentální medicíny, Praha
5 Klinika kardiologie, Institut klinické a experimentální medicíny, Praha

Aim: To present an interesting case of detection of ALmyocardial amyloidosis by cardiac scintigraphy with 99mTc-DPD in a73-year-old patient investigated for exertional dyspnea and heart failure.

Method: Cardiac ultrasound revealed left ventricularhypertrophy with decreased left ventricular systolic function. The patientsubsequently underwent coronary angiography with normal coronary findings.Myocardial amyloidosis was suspected and myocardial scintigraphy with 99mTc-butedronate was indicated. The administered activity of 99mmTc-DPD was 700 MBq intravenously. Planar scintigraphy and chestSPECT were performed 3 hours after intravenous administration of theradiopharmaceutical.

Results: Diffusely increased uptake of 99mTc-DPDin the myocardium of the left and right ventricles was observed. The intensityof tracer uptake corresponded to Perugini scale grade 3. The examination wassuggestive of cardiac amyloidosis. To differentiate transthyretin and ALamyloidosis, we performed immunoelectrophoresis of serum and urinary proteinstogether with assessment of free immunoglobulin light chains. The analysisrevealed marked elevation of free lambda chains and detected paraprotein IgGlambda 6,5 g/L, which confirmed an increased level of free immunoglobulinlight chains. An endomyocardial biopsy confirmed the lambda light chaintype ofmyocardial amyloidosis. A biological therapy, with daratumumab in acombination with bortezomib, cyclophosphamide and dexamethasone, was initiated.

Conclusion: Myocardial scintigraphy with 99mTc-DPDis a highly sensitive method for the diagnosis of transthyretin amyloidcardiomyopathy. However, it can be positive in AL amyloidosis andmonoclonal gammopathy must be evaluated in all subjects with cardiacamyloidosis by means of immunoelectrophoresis of serum and urine together withassessment of free immunoglobulin light chains. An abnormal result of theseassays requires a bioptic verification of amyloid.

Keywords: amyloidosis, heart, 99mTc-DPD, scintigraphy

Published: June 1, 2024  Show citation

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Doležal J, Liška P, Baše M, Radocha J, Buncová M, Kubánek M. Rare detection of AL amyloidosis by 99mTc-DPD cardiac scintigraphy in a 73-year-old patient with exertional dyspnea and heart failure. Ces radiol. 2024;78(2):120-124. doi: 10.55095/CesRadiol2024/016.
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References

  1. Ruberg FL, Berk JL. Transthyretin cardiac amyloidosis. Circulation 2012; 126(10): 1286-1300. Go to original source... Go to PubMed...
  2. Maurer SM, Elliott P, Comenzo R, et al. Addressing common qustions encoutered in the diagnosis and management of cardiac amyloidosis. Circulation 2017; 135(14): 1357-1377. https://doi.org/10.1161/CIRCULATIONAHA.116.024438 Go to original source... Go to PubMed...
  3. Siddiqi OK, Ruberg FL. Cardiac Amyloidosis: an update on pathophysiology, diagnosis and treatment. Trends Cardiovasc Med. 2018; 28(1): 10-21. doi:10.1016/j.tcm.2017.007.004 Go to original source... Go to PubMed...
  4. Torres-Courchoud I, Martínez-Gil R, Aibar-Arregui MA, et al. Cardiac Involvement Secondary to a Familial Form of Transthyretin Amyloidosis Resulting From the Glu54Gln Mutation. Rev Esp Cardiol. 2017; 70(4): 297-299. doi:10.1016/j.rec.2016.07.001 Go to original source... Go to PubMed...
  5. Gertz MA, Benson MD, Dyck PJ, et al. Diagnosis, prognosis and therapy of transthyretin amyloidosis. J Am Coll Cardiol. 2015; 66(21): 2451-2466. doi:10.1016/j.jacc.2015.09.075 Go to original source... Go to PubMed...
  6. Pika T, Vymětal J, Metelka R, et al. Postižení srdce při AL amyloidóze. Interní Med. 2008; 10(10): 466-469.
  7. Gillmore JD, Damy T, Fontana M, et al. A new staging system for cardiac transthyretin amyloidosis. Eur Heart J. 2017; 39(30): 2799-2806. Go to original source... Go to PubMed...
  8. Cornwell GG 3rd, Murdoch WL, Kyle RA, et al. Frequency and distribution of senile cardiovascular amyloid. A clinicopathological correlation. Am J Med. 1983; 75(4): 618-623. Go to original source... Go to PubMed...
  9. Dubrey SW, Cha K, Anderson J, et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. Q J Med. 1998; 91(2): 141-157. Go to original source... Go to PubMed...
  10. Perugini E, Guidalotti PL, Salvi F, et al. Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol. 2005; 46(6): 1076-1084. Go to original source... Go to PubMed...
  11. Hutt DF, Quigley AM, Page J, et al. Utility and limitations of 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy in systemic amyloidosis. Eur Heart J Cardiovasc Imaging 2014; 15(11): 1289-1298. doi:10.1093/ehjci/jeu107 [Epub 2014 Jun 16]. Go to original source... Go to PubMed...
  12. Quarta CC, Zheng J, Hutt D, et al. 99mTc-DPD scintigraphy in immunoglobulin light chain (AL) cardiac amyloidosis. Eur Heart J Cardiovascular Imaging 2021; 22(11): 1304-1311. Go to original source... Go to PubMed...
  13. Andrikopolou E, et al. Nuclear Imaging of cardiac amyloidosis. J Nucl Cardiol. 2019; 26(2): 505-508. Go to original source... Go to PubMed...
  14. www.asnc.org/Files/Practice%20Resources/Practice%20Points/ASNC%20Practice%20Point-99mTechnetiumPyrophosphateImaging2016.pdf
  15. Zoller M., Hajnos G., Burkhard R, et al. 99mTc-DPD-scintigraphy with intense uptake in fat tissue caused by AL-amyloidosis. Eur J Nucl Med Mol Imging 2016; 43(12): 2269-2270. Go to original source... Go to PubMed...
  16. Persia-Paulino YR, Cuevas-Perez J, Fernandez-Asensio R, et al. Unusual high 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) tracer deposition on a heart scintigraphy in a patient with AL amyloidosis: A case report. J Nucl Cardiol. 2021; 28(3): 1117-1125. Go to original source... Go to PubMed...

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