Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis.

Autor:	Chacko L; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Martone R; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK.; Department of Heart, Lung and Vessels, Tuscan Regional Amyloid Center, Careggi University Hospital, Largo Brambilla 3, Florence 50134, Italy., Bandera F; Heart Failure Unit, Cardiology University Department, IRCCS Policlinico San Donato, Piazza Malan, 1, San Donato Milanese, Milan 20097, Italy.; Department for Biomedical Sciences for Health, University of Milano, Via Luigi Mangiagalli, 31, Milan 20133, Italy., Lane T; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Martinez-Naharro A; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Boldrini M; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Rezk T; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Whelan C; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Quarta C; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Rowczenio D; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Gilbertson JA; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Wongwarawipat T; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Lachmann H; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Wechalekar A; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Sachchithanantham S; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Mahmood S; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Marcucci R; Department of Experimental and Clinical Medicine, Careggi University Hospital, University of Florence, Largo Brambilla 3, Florence 50134, Italy., Knight D; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Hutt D; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Moon J; Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit, and the Inherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.; Institute of Cardiovascular Science, University College London, Gower Street, London WC1E 6BT, UK., Petrie A; Biostatistics Unit, UCL Eastman Dental Institute, University College London, 256 Grays Inn Road, London WC1X 8LD, UK., Cappelli F; Department of Heart, Lung and Vessels, Tuscan Regional Amyloid Center, Careggi University Hospital, Largo Brambilla 3, Florence 50134, Italy., Guazzi M; Heart Failure Unit, Cardiology University Department, IRCCS Policlinico San Donato, Piazza Malan, 1, San Donato Milanese, Milan 20097, Italy.; Department for Biomedical Sciences for Health, University of Milano, Via Luigi Mangiagalli, 31, Milan 20133, Italy., Hawkins PN; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Gillmore JD; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK., Fontana M; National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK.
Jazyk:	angličtina
Zdroj:	European heart journal [Eur Heart J] 2020 Apr 07; Vol. 41 (14), pp. 1439-1447.
DOI:	10.1093/eurheartj/ehz905
Abstrakt:	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. (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
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