Transthyretin amyloid cardiomyopathy among patients with heart failure and preserved ejection fraction: the AMY score.
AMY score
ATTRwt
Cardiac amyloidosis
Endomyocardial biopsy
Heart failure with preserved ejection fraction
Screening
Journal
ESC heart failure
ISSN: 2055-5822
Titre abrégé: ESC Heart Fail
Pays: England
ID NLM: 101669191
Informations de publication
Date de publication:
13 Apr 2024
13 Apr 2024
Historique:
revised:
03
03
2024
received:
12
07
2023
accepted:
15
03
2024
medline:
13
4
2024
pubmed:
13
4
2024
entrez:
13
4
2024
Statut:
aheadofprint
Résumé
Transthyretin 'wild-type' amyloid cardiomyopathy (ATTRwt-CM) is a differential diagnosis of heart failure with preserved ejection fraction (HFpEF). The clinical work-up for ATTRwt-CM is challenging. Considering a combination of clinical variables specific for ATTRwt-CM might aid in identifying patients at risk. Sixty patients (78 ± 6 years, 8% female) were diagnosed with ATTRwt-CM by endomyocardial biopsy. Preserved ejection fraction (LVEF >45%) was present in 41 of the patients. Those were 1:1 propensity score age- and sex-matched to a cohort of patients with HFpEF. ATTRwt-CM patients had less obesity (P = 0.01) and higher septal thickness (IVSd, P < 0.01) as well as more diastolic dysfunction (E/e', P < 0.01). On multivariable regression IVSd > 14 mm, E/e' > 14 and absence of obesity (P > 0.01 for all) were identified as predictors for ATTRwt-CM. A weighted point-based score was derived with IVSd > 14 mm = 1 point; absence of obesity = 2 points; and E/e' > 14 = 3 points. Area under the curve (AUC) for the summation score was 0.91 (0.84-0.97, P < 0.01) and a score of more than 3 points predicted ATTRwt-CM with good sensitivity (78%) and specificity (90%). The score was validated in an external cohort of 142 patients with ATTRwt-CM and 419 HFpEF patients showing sufficient accuracy (AUC 0.91, 0.88-0.94, P < 0.01). A value greater than 3 points demonstrated a high sensitivity (93%) and a negative predictive value of 97%. A score based on basic clinical and echocardiographic features helps to distinguish ATTRwt-CM from typical HFpEF. This could facilitate the diagnostic work-up for these patients and enable earlier disease screening on a large scale.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Else-Kröner-Fresenius-Stiftung, Bad Homburg, Germany
Informations de copyright
© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
Références
Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, et al. Systemic cardiac amyloidoses: Disease profiles and clinical courses of the 3 main types. Circulation 2009;120:1203‐1212. doi:10.1161/CIRCULATIONAHA.108.843334
Merlini G, Seldin DC, Gertz MA. Amyloidosis: Pathogenesis and new therapeutic options. J Clin Oncol 2011;29:1924‐1933. doi:10.1200/JCO.2010.32.2271
Lie JT, Hammond PI. Pathology of the senescent heart: anatomic observations on 237 autopsy studies of patients 90 to 105 years old. Mayo Clin Proc 1988;63:552‐564. doi:10.1016/s0025‐6196(12)64885‐x
González‐López E, Gallego‐Delgado M, Guzzo‐Merello G, de Haro‐del Moral FJ, Cobo‐Marcos M, Robles C, et al. Wild‐type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J 2015;36:2585‐2594. doi:10.1093/eurheartj/ehv338
Gertz MA, Benson MD, Dyck PJ, Grogan M, Coelho T, Cruz M, et al. Diagnosis, prognosis, and therapy of transthyretin amyloidosis. J Am Coll Cardiol 2015;66:2451‐2466. doi:10.1016/j.jacc.2015.09.075
Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington‐Cruz M, et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med 2018;379:1007‐1016. doi:10.1056/NEJMoa1805689
Davies DR, Redfield MM, Scott CG, et al. A simple score to identify increased risk of transthyretin amyloid cardiomyopathy in heart failure with preserved ejection fraction. JAMA doi:10.1001/jamacardio.2022.1781
Besler C, Rommel KP, Kresoja KP, Mörbitz J, Kirsten H, Scholz M, et al. Evaluation of phosphodiesterase 9A as a novel biomarker in heart failure with preserved ejection fraction. ESC Hear Fail 2021;8:1861‐1872. doi:10.1002/ehf2.13327
Scholz M, Henger S, Beutner F, Teren A, Baber R, Willenberg A, et al. Cohort profile: The Leipzig research center for civilization diseases‐heart study (LIFE‐heart). Int J Epidemiol 2020;49:1439‐1440H. doi:10.1093/ije/dyaa075
Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JGF, Coats AJS, et al. 2016 ESC guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2016;37:2129‐2200m. doi:10.1093/eurheartj/ehw128
Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 2016;133:2404‐2412. doi:10.1161/CIRCULATIONAHA.116.021612
Yumuk V, Tsigos C, Fried M, Schindler K, Busetto L, Micic D, et al. European guidelines for obesity management in adults. Obes Facts 2015;8:402‐424. doi:10.1159/000442721
Cosentino F, Grant PJ, Aboyans V, Bailey CJ, Ceriello A, Delgado V, et al. 2019 ESC guidelines on diabetes, pre‐diabetes, and cardiovascular diseases developed in collaboration with the EASD. Eur Heart J 2020;41:255‐323. doi:10.1093/eurheartj/ehz486
Vahanian A, Beyersdorf F, Praz F, Milojevic M, Baldus S, Bauersachs J, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease: developed by the task force for the management of valvular heart disease of the European Society of Cardiology (ESC) and the European Association for Cardio‐Thoracic Surgery (EACTS). Rev Esp Cardiol (Engl Ed) 2022;75:524. doi:10.1016/j.rec.2022.05.006
Lang RM, Badano LP, Mor‐Avi V, Afilalo J, Armstrong A, Ernande L, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the American society of echocardiography and the European association of cardiovascular imaging. Eur Heart J Cardiovasc Imaging 2015;16:233‐271. doi:10.1093/ehjci/jev014
Gillmore JD, Damy T, Fontana M, Hutchinson M, Lachmann HJ, Martinez‐Naharro A, et al. A new staging system for cardiac transthyretin amyloidosis. Eur Heart J 2018;39:2799‐2806. doi:10.1093/eurheartj/ehx589
Valentini F, Anselmi F, Metra M, Cavigli L, Giacomin E, Focardi M, et al. Diagnostic and prognostic value of low QRS voltages in cardiomyopathies: old but gold. Eur J Prev Cardiol 2022;29:1177‐1187. doi:10.1093/eurjpc/zwaa027
DeLong ER, DeLong DM, Clarke‐Pearson DL. Comparing the areas under two or more correlated receiver operating characteristic curves: A nonparametric approach. Biometrics 1988;44:837‐845. doi:10.2307/2531595
Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J, et al. Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2‐macroglobulin and tau: a population‐based autopsy study. Ann Med 2008;40:232‐239. doi:10.1080/07853890701842988
Stern LK. Cardiac amyloidosis treatment. 2022;18:59‐72. doi:10.14797/mdcvj.1050
Maurer MS, Grogan DR, Judge DP, Mundayat R, Packman J, Lombardo I, et al. Tafamidis in transthyretin amyloid cardiomyopathy: Effects on transthyretin stabilization and clinical outcomes. Circ Heart Fail 2015;8:519‐526. doi:10.1161/CIRCHEARTFAILURE.113.000890
Verwerft J, Soens L, Wynants J, Meysman M, Jogani S, Plein D, et al. Heart failure with preserved ejection fraction: Relevance of a dedicated dyspnoea clinic. Eur Heart J 2023;44:1544‐1556.
Garcia‐Pavia P, Rapezzi C, Adler Y, Arad M, Basso C, Brucato A, et al. Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC working group on myocardial and pericardial diseases. Eur Heart J 2021;42:1554‐1568.
González‐López E, Gagliardi C, Dominguez F, Quarta CC, de Haro‐del Moral FJ, Milandri A, et al. Clinical characteristics of wild‐type transthyretin cardiac amyloidosis: Disproving myths. Eur Heart J 2017;38:1895‐1904.
Chacko L, Martone R, Bandera F, Lane T, Martinez‐Naharro A, Boldrini M, et al. Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis. Eur Heart J 2020;41:1439‐1447a. doi:10.1093/eurheartj/ehz905
Oghina S, Bougouin W, Bézard M, Kharoubi M, Komajda M, Cohen‐Solal A, et al. The impact of patients with cardiac amyloidosis in HFpEF trials. JACC Hear Fail 2021;9:169‐178. doi:10.1016/j.jchf.2020.12.005
Duca F, Snidat A, Binder C, Rettl R, Dachs TM, Seirer B, et al. Hemodynamic profiles and their prognostic relevance in cardiac amyloidosis. J Clin Med 2020;9:1093. doi:10.3390/jcm9041093
Hsu S, Fang JC, Borlaug BA. Hemodynamics for the heart failure clinician: A state‐of‐the‐art review. J Card Fail 2022;28:133‐148. doi:10.1016/j.cardfail.2021.07.012
Borlaug BA, Paulus WJ. Heart failure with preserved ejection fraction: pathophysiology, diagnosis, and treatment. Eur Heart J 2011;32:670‐679. doi:10.1093/eurheartj/ehq426
Kittleson MM, Maurer MS, Ambardekar AV, Bullock‐Palmer RP, Chang PP, Eisen HJ, et al. Cardiac amyloidosis: Evolving diagnosis and management: A scientific statement from the American Heart Association. Circulation 2020;E7–E22:e7‐e22. doi:10.1161/CIR.0000000000000792