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Noninvasive Risk Stratification of Patients With Transthyretin Amyloidosis

2014; Elsevier BV; Volume: 7; Issue: 5 Linguagem: Inglês

10.1016/j.jcmg.2014.03.002

1936-878X

Arnt V. Kristen, Katrin Scherer, Sebastian J. Buss, Fabian aus dem Siepen, Sabine Haufe, Ralf Bauer, Katrin Hinderhofer, Evangelos Giannitsis, Stefan E. Hardt, Uwe Haberkorn, Hugo A. Katus, Henning Steen,

Cardiac electrophysiology and arrhythmias

We sought to evaluate noninvasive parameters by electrocardiography, echocardiography, technetium-99m–3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy, and cardiac magnetic resonance for the prediction of all-cause mortality in patients with cardiac transthyretin amyloidosis (ATTR). ATTR may present with highly variable symptoms, including polyneuropathy and cardiomyopathy, the latter being associated with a poor outcome. However, data on noninvasive risk stratification of ATTR are limited. A total of 70 patients with ATTR were evaluated by echocardiography, cardiac biomarkers, and 99mTc-DPD scintigraphy. Cardiac magnetic resonance was performed in 30 patients. Echocardiographic findings and plasma levels of biomarkers were correlated with results of quantitative analysis of scintigraphy using a region-of-interest technique (whole-body as well as heart tracer retention). Receiver-operating characteristic (ROC) analysis was performed to calculate a cutoff value of 99mTc-DPD scintigraphy for heart retention for the diagnosis of cardiac amyloid involvement with the highest sensitivity and specificity. Univariate and multivariate analyses were performed in patients with cardiac involvement (n = 60) to determine noninvasive predictors of all-cause mortality. Scintigraphy findings correlated with morphological (interventricular septum thickness, left ventricular hypertrophy index) as well as functional (mitral annular systolic velocity, mitral/tricuspid annular plane systolic excursion) findings, cardiac biomarkers, renal function, and late gadolinium enhancement. The ROC-derived cutoff for the detection of cardiac amyloidosis by scintigraphic heart tracer retention was 4.8%. Univariate Cox regression revealed N-terminal pro–B-type natriuretic peptide, troponin T, mitral annular plane systolic excursion, and left ventricular hypertrophy index as predictors of all-cause mortality. However, on multivariate analysis, troponin T remained the only independent predictor of survival. The ROC-derived cutoff value of troponin T predicting all-cause mortality with the highest sensitivity (80.0%) and specificity (68.7%) was 0.0375 ng/l. Quantitative analysis of tracer retention is capable of characterizing the severity of cardiac involvement in ATTR. By multivariate analysis, troponin T remained the only independent predictor of survival.