Skeletal scintigraphy indicates disease severity of cardiac involvement in patients with senile systemic amyloidosis

• Published in: International journal of cardiology 2013-04, Vol.164 (2), p.179-184
• Main Authors: Kristen, Arnt V, Haufe, Sabine, Schonland, Stefan O, Hegenbart, Ute, Schnabel, Philipp A, Röcken, Christoph, Hardt, Stefan, Lohse, Peter, Ho, Anthony D, Haberkorn, Uwe, Dengler, Thomas J, Altland, Klaus, Katus, Hugo A
• Format: Article
• Language: English
• Subjects: 99mTc-DPD scintigraphy; Aged; Aged, 80 and over; Amyloidosis; Amyloidosis - diagnostic imaging; Amyloidosis - epidemiology; Biological and medical sciences; Cardiology. Vascular system; Cardiovascular; Cohort Studies; Female; Heart; Heart - diagnostic imaging; Heart failure; Heart failure, cardiogenic pulmonary edema, cardiac enlargement; Humans; Left ventricular longitudinal function; Male; Medical sciences; Metabolic diseases; Middle Aged; Other metabolic disorders; Plaque, Amyloid - diagnostic imaging; Plaque, Amyloid - epidemiology; Radionuclide Imaging; Risk assessment; Senile systemic amyloidosis; Severity of Illness Index; Survival; Ventricular Dysfunction, Left - diagnostic imaging; Ventricular Dysfunction, Left - epidemiology
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2011.06.123

Abstract Background Senile systemic amyloidosis (SSA) is a common aging phenomenon in the elderly population. Nevertheless, pre-mortem diagnosis of SSA is rare. Thus, data on clinical characterization and disease severity are limited. Methods 36 consecutive SSA patients (71.6 [64.7–82.7] years) were evaluated by electrocardiography, echocardiography, laboratory tests, and99m Technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (99m Tc-DPD) scintigraphy (n = 20). Results In addition to cardiac involvement, amyloid deposition was found in rectum (n = 6), peripheral nerves (n = 2), and urinary bladder (n = 2). Five patients showed low voltage pattern. Thickness of interventricular septum (IVS) was 20 [12–27] mm. LV longitudinal function was diminished (TDI-s 5 [3–11] cm/s; MAPSE 6.5 [2.5–19] mm; TAPSE 12.5 [2–24] mm). LV systolic function (LV-EF < 45%) was markedly decreased in 19 patients. Plasma levels of troponin T (0.05 [0.01–0.23] µg/L) and NT-proBNP (4318 [205–16597] ng/L) were elevated.99m Tc-DPD heart retention was 7.8 [2.4–11.0]% and correlated with MAPSE (ρ = − 0.716; p = 0.0018), TAPSE (ρ = − 0.491; p < 0.05), and IVS (ρ = 0.556; p = 0.0153). Heart-to-body ratio correlated with MAPSE (ρ = − 0.771; p = 0.0018), IVS (ρ = 0.603; p = 0.0086). Twelve patients died during follow-up of 27.4 [0.1–106.2] months. Exclusively99m Tc-DPD heart retention, diastolic dysfunction and in trend MAPSE were associated with patient's outcome. Interestingly, risk predictors that were well established in patients with AL amyloidosis were not predictive for survival in patients with SSA. Conclusions This study gave first evidence that99m Tc-DPD HR may be capable to display the extent of cardiac amyloid deposition. Moreover, this study suggested that99m Tc-DPD HR, diastolic dysfunction and in trend MAPSE are associated with poor outcome. Nevertheless, these findings need to be established in a larger prospective trial.