Martin Nicol1, Mathilde Baudet1, Stephanie Brun2, Stephanie Harel3, Bruno Royer3, Marguerite Vignon4, Olivier Lairez2, David Lavergne5, Arnaud Jaccard5, David Attias6, Laurent Macron6, Etienne Gayat7,8, Alain Cohen-Solal1,8, Bertrand Arnulf2,8, Damien Logeart1,8. 1. Cardiology Department, Hopital Lariboisiere, 2 rue Ambroise Paré, 75010 Paris, France. 2. Cardiology Department, Hopital Rangueil, 2 rue Viguerie, 31300 Toulouse, France. 3. Immuno-Hematology Department, Hopital Saint Louis, 2 avenue Claude Vellefaux, 75010 Paris, France. 4. Hematology Department, Hopital Cochin, 27 rue du Faubourg Saint Jacques, 75014 Paris, France. 5. Hematology Department, Hopital Dupuytren, 2 avenue Martin Luther King, 87042 Limoges, France. 6. Cardiology Department, Centre cardiologique du Nord, 32 rue des Moulins Gémeaux, 93200 Saint Denis, France. 7. Anesthesiology Department, Hopital Lariboisiere, 2 rue Ambroise Paré, 75010 Paris, France. 8. Université de Paris, 10 avenue de Verdun, 75010 Paris, France.
Abstract
AIMS: Early diagnosis of cardiac involvement is a key issue in the management of AL amyloidosis. Our objective was to establish a diagnostic score of cardiac involvement in AL amyloidosis and to compare it with the current consensus criteria [i.e. left ventricular hypertrophy >12 mm and N-terminal pro b-type natriuretic peptide (NT-proBNP) >332 ng/L]. METHODS AND RESULTS: We carried out a prospective and multicenter study on AL amyloidosis patients who underwent cardiac evaluation including clinical examination, electrocardiography (ECG), cardiac biomarkers, transthoracic echocardiography (TTE), and cardiac magnetic resonance imaging (CMR). Cardiac involvement was based on CMR and/or endomyocardial biopsy. In a derivation cohort of 114 patients (82 with cardiac involvement), the highest diagnostic accuracy was observed with NT-proBNP and troponin blood levels, TTE-derived global longitudinal strain (LS), and apical to basal LS gradient. By using multivariate analysis, we established a diagnostic score including global LS ≥-17% (1 point), apical/(basal + median) LS ≥0.90 (1 point), and troponin T >35 ng/L (1 point). A score >1 was associated with sensitivity of 94% and specificity of 97%, an area under the curve of 0.98 [95% confidence interval (CI) 0.93-0.99] as well as a net reclassification index of 0.39 (95% CI 0.28-0.46) when compared with consensus criteria. In a validation cohort of 73 AL amyloidosis patients, the area under the receiver operating characteristic curve of the diagnostic score was 0.97 (95% CI 0.90-0.99). CONCLUSION: Combining T troponin blood levels and two echo-derived strain parameters leads to very high accuracy for diagnosing cardiac involvement in AL amyloid patients. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: Early diagnosis of cardiac involvement is a key issue in the management of AL amyloidosis. Our objective was to establish a diagnostic score of cardiac involvement in AL amyloidosis and to compare it with the current consensus criteria [i.e. left ventricular hypertrophy >12 mm and N-terminal pro b-type natriuretic peptide (NT-proBNP) >332 ng/L]. METHODS AND RESULTS: We carried out a prospective and multicenter study on AL amyloidosispatients who underwent cardiac evaluation including clinical examination, electrocardiography (ECG), cardiac biomarkers, transthoracic echocardiography (TTE), and cardiac magnetic resonance imaging (CMR). Cardiac involvement was based on CMR and/or endomyocardial biopsy. In a derivation cohort of 114 patients (82 with cardiac involvement), the highest diagnostic accuracy was observed with NT-proBNP and troponin blood levels, TTE-derived global longitudinal strain (LS), and apical to basal LS gradient. By using multivariate analysis, we established a diagnostic score including global LS ≥-17% (1 point), apical/(basal + median) LS ≥0.90 (1 point), and troponin T >35 ng/L (1 point). A score >1 was associated with sensitivity of 94% and specificity of 97%, an area under the curve of 0.98 [95% confidence interval (CI) 0.93-0.99] as well as a net reclassification index of 0.39 (95% CI 0.28-0.46) when compared with consensus criteria. In a validation cohort of 73 AL amyloidosispatients, the area under the receiver operating characteristic curve of the diagnostic score was 0.97 (95% CI 0.90-0.99). CONCLUSION: Combining T troponin blood levels and two echo-derived strain parameters leads to very high accuracy for diagnosing cardiac involvement in AL amyloid patients. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Asan Agibetov; Benjamin Seirer; Theresa-Marie Dachs; Matthias Koschutnik; Daniel Dalos; René Rettl; Franz Duca; Lore Schrutka; Hermine Agis; Renate Kain; Michela Auer-Grumbach; Christina Binder; Julia Mascherbauer; Christian Hengstenberg; Matthias Samwald; Georg Dorffner; Diana Bonderman Journal: J Clin Med Date: 2020-05-03 Impact factor: 4.241