World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM)

被引:27
作者
Brito, Dulce [1 ]
Albrecht, Fabiano Castro [2 ]
Perez De Arenaza, Diego [3 ]
Bart, Nicole [4 ]
Better, Nathan [5 ,6 ]
Carvajal-Juarez, Isabel [7 ]
Conceicao, Isabel [8 ,9 ]
Damy, Thibaud [10 ,11 ,12 ]
Dorbala, Sharmila [13 ,14 ,15 ,16 ]
Fidalgo, Jean-Christophe [17 ]
Garcia-Pavia, Pablo [18 ,19 ]
Ge, Junbo [20 ]
Gillmore, Julian D. [21 ]
Grzybowski, Jacek [22 ]
Obici, Laura [23 ]
Pinero, Daniel [24 ]
Rapezzi, Claudio [25 ]
Ueda, Mitsuharu [26 ]
Pinto, Fausto J. [1 ,27 ]
机构
[1] Univ Lisbon, Dept Cardiol, Ctr Hosp Univ Lisboa Norte, CAML,CCUL RISE,Fac Med, Lisbon, Portugal
[2] Cardiac Amyloidosis Ctr Dante Pazzanese Inst, Dante Pazzanese Inst Cardiol, Sao Paulo, Brazil
[3] Hosp Italiano Buenos Aires, Cardiol Dept, Buenos Aires, DF, Argentina
[4] Univ New South Wales, Victor Chang Cardiac Res Inst, St Vincents Hosp, Sydney, NSW, Australia
[5] Monash Univ, Royal Melbourne Hosp, Cabrini Hlth, Parkville, Vic, Australia
[6] Univ Melbourne, Melbourne, Vic, Australia
[7] Inst Nacl Cardiol Ignacio Chavez, Mexico City, DF, Mexico
[8] CHULN Hosp Santa Maria, Dept Neurosci & Mental Hlth, Lisbon, Portugal
[9] Univ Lisboa Portugal, Fac Med, Ctr Estudos Egas Moniz, Lisbon, Portugal
[10] CHU Henri Mondor, Dept Cardiol, DHU A TVB, AP HP,INSERM,U955, Creteil, France
[11] UPEC, Creteil, France
[12] GRC Amyloid Res Inst, Referral Ctr Cardiac Amyloidosis, Reseau Amylose, Creteil, France
[13] Harvard Med Sch, Div Nucl Med & Mol Imaging, Dept Radiol, Brigham & Womens Hosp, Boston, MA 02115 USA
[14] Harvard Med Sch, Cardiac Amyloidosis Program, Cardiovasc Div, Dept Med,Brigham & Womens Hosp, Boston, MA 02115 USA
[15] Harvard Med Sch, CV Imaging Program, Cardiovasc Div, Brigham & Womens Hosp, Boston, MA 02115 USA
[16] Harvard Med Sch, Dept Radiol, Brigham & Womens Hosp, Boston, MA 02115 USA
[17] Amyloidosis Alliance Voice Patients, Boston, MA USA
[18] Hosp Univ Puerta Hierro Majadahonda, IDIPHISA, CIBERCV, Madrid, Spain
[19] Ctr Nacl Invest Cardiovasc CNIC, Madrid, Spain
[20] Fudan Univ, Shanghai Inst Cardiovasc Dis, Zhongshan Hosp, Dept Cardiol, Shanghai, Peoples R China
[21] UCL, Natl Amyloidosis Ctr, Royal Free Campus, London, England
[22] Natl Inst Cardiol, Dept Cardiomyopathy, Warsaw, Poland
[23] Fdn IRCCS Policlin San Matteo, Amyloidosis Res & Treatment Ctr, Pavia, Italy
[24] Univ Buenos Aires, Buenos Aires, DF, Argentina
[25] Univ Ferrara, Cardiovasc Inst, Ferrara, Italy
[26] Kumamoto Univ, Grad Sch Med Sci, Dept Neurol, Kumamoto, Japan
[27] Univ Lisbon, Fac Med, Lisbon, Portugal
关键词
Heart Failure; Amyloidosis; Transthyretin amyloid cardiomyopathy (ATTR-CM); diagnosis; treatment; patients' perspective; CARDIOVASCULAR MAGNETIC-RESONANCE; QUALITY-OF-LIFE; CARDIAC AMYLOIDOSIS; BONE-SCINTIGRAPHY; AORTIC-STENOSIS; INTRACARDIAC THROMBOSIS; PROGNOSTIC-SIGNIFICANCE; RISK STRATIFICATION; ETIOLOGIC DIAGNOSIS; POSITION STATEMENT;
D O I
10.5334/gh.1262
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive and fatal condition that requires early diagnosis, management, and specific treatment. The availability of new disease-modifying therapies has made successful treatment a reality. Transthyretin amyloid cardiomyopathy can be either age-related (wild-type form) or caused by mutations in the TTR gene (genetic, hereditary forms). It is a systemic disease, and while the genetic forms may exhibit a variety of symptoms, a predominant cardiac phenotype is often present. This document aims to provide an overview of ATTR-CM amyloidosis focusing on cardiac involvement, which is the most critical factor for prognosis. It will discuss the available tools for early diagnosis and patient management, given that specific treatments are more effective in the early stages of the disease, and will highlight the importance of a multidisciplinary approach and of specialized amyloidosis centres. To accomplish these goals, the World Heart Federation assembled a panel of 18 expert clinicians specialized in TTR amyloidosis from 13 countries, along with a representative from the Amyloidosis Alliance, a patient advocacy group. This document is based on a review of published literature, expert opinions, registries data, patients' perspectives, treatment options, and ongoing developments, as well as the progress made possible via the existence of centres of excellence. From the patients' perspective, increasing disease awareness is crucial to achieving an early and accurate diagnosis. Patients also seek to receive care at specialized amyloidosis centres and be fully informed about their treatment and prognosis.
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页数:47
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