Data di Pubblicazione:
2020
Citazione:
Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis / L. Chacko, R. Martone, F. Bandera, T. Lane, A. Martinez-Naharro, M. Boldrini, T. Rezk, C. Whelan, C. Quarta, D. Rowczenio, J.A. Gilbertson, T. Wongwarawipat, H. Lachmann, A. Wechalekar, S. Sachchithanantham, S. Mahmood, R. Marcucci, D. Knight, D. Hutt, J. Moon, A. Petrie, F. Cappelli, M. Guazzi, P.N. Hawkins, J.D. Gillmore, M. Fontana. - In: EUROPEAN HEART JOURNAL. - ISSN 0195-668X. - 41:14(2020 Apr 07), pp. 1439-1447.
Abstract:
Aims: Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. We sought to characterize the structural and functional echocardiographic phenotype across the spectrum of wild-type (wtATTR-CM) and hereditary (hATTR-CM) transthyretin cardiomyopathy and the echocardiographic features predicting prognosis. Methods and results: We studied 1240 patients with ATTR-CM who underwent prospective protocolized evaluations comprising full echocardiographic assessment and survival between 2000 and 2019, comprising 766 with wtATTR-CM and 474 with hATTR-CM, of whom 314 had the V122I variant and 127 the T60A variant. At diagnosis, patients with V122I-hATTR-CM had the most severe degree of systolic and diastolic dysfunction across all echocardiographic parameters and patients with T60AhATTR-CM the least; patients with wtATTR-CM had intermediate features. Stroke volume index, right atrial area index, longitudinal strain, and E/e' were all independently associated with mortality (P < 0.05 for all). Severe aortic stenosis (AS) was also independently associated with prognosis, conferring a significantly shorter survival (median survival 22 vs. 53 months, P = 0.001). Conclusion: The three distinct genotypes present with varying degrees of severity. Echocardiography indicates a complex pathophysiology in which both systolic and diastolic function are independently associated with mortality. The presence of severe AS was independently associated with significantly reduced patient survival.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Amyloidosis; Aortic stenosis; Cardiomyopathy; Echocardiography; Prognosis; Transthyretin
Elenco autori:
L. Chacko, R. Martone, F. Bandera, T. Lane, A. Martinez-Naharro, M. Boldrini, T. Rezk, C. Whelan, C. Quarta, D. Rowczenio, J.A. Gilbertson, T. Wongwarawipat, H. Lachmann, A. Wechalekar, S. Sachchithanantham, S. Mahmood, R. Marcucci, D. Knight, D. Hutt, J. Moon, A. Petrie, F. Cappelli, M. Guazzi, P.N. Hawkins, J.D. Gillmore, M. Fontana
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