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CARDIAC IMAGING

Noninvasive Etiologic Diagnosis of Cardiac Amyloidosis Using ^99mTc-3,3-Diphosphono-1,2-Propanodicarboxylic Acid Scintigraphy

Enrica Perugini, MD^_*, Pier Luigi Guidalotti, MD, Fabrizio Salvi, MD, Robin M.T. Cooke, MA^_*, Cinzia Pettinato, MD, Letizia Riva, MD^_*, Ornella Leone, MD, Mohsen Farsad, MD, Paolo Ciliberti, MD^_*, Letizia Bacchi-Reggiani, MSc, MBiostat^_*, Francesco Fallani, MD^_*, Angelo Branzi, MD^_* and Claudio Rapezzi, MD^_*,_*

^_* Institute of Cardiology
Nuclear Medicine Unit
Department of Pathology, University of Bologna and S. Orsola-Malpighi Hospital, Bologna, Italy
Department of Neurology, Ospedale Bellaria, Bologna, Italy

Manuscript received March 4, 2005; revised manuscript received May 18, 2005, accepted May 24, 2005.

^_* Reprint requests and correspondence: Prof. Claudio Rapezzi, Istituto di Cardiologia, Policlinico S. Orsola-Malpighi, via Massarenti 9, 40138 Bologna, Italy (Email: crapezzi{at}aosp.bo.it).

OBJECTIVES: We investigated the diagnostic accuracy of ^99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (^99mTc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis.

BACKGROUND: Differential diagnosis between TTR-related and AL amyloidosis is often complex and time-consuming.

METHODS: Patients under routine observation with TTR-related/AL systemic amyloidosis and echocardiographic evidence of cardiac involvement were studied with ^99mTc-DPD scintigraphy.

RESULTS: Patients with cardiac involvement of TTR-related (group A; n = 15) and AL (group B; n = 10) etiology were comparable for left ventricular mass and renal function. Heart and heart/whole-body tracer retention were significantly higher (p < 0.05) in group A as compared with group B and with 10 unaffected controls. At visual scoring, cardiac ^99mTc-DPD uptake was present in all group A patients and absent in all group B patients; thus, using genotyping/immunohistochemistry as the reference technique, the accuracy of ^99mTc-DPD scintigraphy for distinction of TTR-related and AL etiology was 100%. Cardiac ^99mTc-DPD uptake was also absent among unaffected controls. Using echocardiography as the reference standard for recognition of cardiac involvement, sensitivity and specificity of scintigraphy were both 100% for group A patients; in group B, sensitivity was 0% and specificity was 100% (accuracy, 50%). Eleven patients with myocardial ^99mTc-DPD uptake underwent ^99mTc-methylene diphosphonate (^99mTc-MDP) scintigraphy; all patients showed a ^99mTc-MDP myocardial visual score of 0.

CONCLUSIONS: Etiology is a third major cause—in addition to type of organ-involved (soft-tissue/heart) and tracer type—of scintigraphic variability in cardiac amyloidosis. This is a highly relevant consideration for future studies. We conclude that ^99mTc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.

Abbreviations and Acronyms   AL = monoclonal immunoglobulin light-chain   CA = cardiac amyloidosis   LV = left ventricle/ventricular   OLT = orthotopic liver transplantation   ROI = region of interest   SPECT = single-photon emission computed tomography   99mTc-DPD = 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid   99mTc-MDP = 99mTc-methylene diphosphonate   TTR = transthyretin

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