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Sex-Related Risk of Cardiac Involvement in Hereditary Transthyretin Amyloidosis: Insights From THAOS.
Caponetti, Angelo Giuseppe; Rapezzi, Claudio; Gagliardi, Christian; Milandri, Agnese; Dispenzieri, Angela; Kristen, Arnt V; Wixner, Jonas; Maurer, Mathew S; Garcia-Pavia, Pablo; Tournev, Ivailo; Planté-Bordeneuve, Violaine; Chapman, Douglass; Amass, Leslie.
Afiliação
  • Caponetti AG; Department of Experimental, Diagnostic, and Specialty Medicine, University of Bologna, Italy.
  • Rapezzi C; Cardiological Center, University of Ferrara, Ferrara, Italy; Maria Cecilia Hospital, GVM Care and Research, Cotignola, Emilia-Romagna, Italy. Electronic address: Claudio.rapezzi@unibo.it.
  • Gagliardi C; Department of Experimental, Diagnostic, and Specialty Medicine, University of Bologna, Italy.
  • Milandri A; Department of Cardiology, Bentivoglio Hospital, Bentivoglio, Bologna, Italy.
  • Dispenzieri A; Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
  • Kristen AV; Department of Cardiology, Angiology, Respiratory Medicine, Medical University of Heidelberg, Heidelberg, Germany.
  • Wixner J; Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
  • Maurer MS; Columbia University College of Physicians and Surgeons, New York, New York, USA.
  • Garcia-Pavia P; Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares, Madrid, Spain; Universidad Francisco de Vitoria, Pozuelo de Alarcon, Madrid, Spain.
  • Tournev I; Clinic of Nervous Diseases, University Hospital Aleksandrovska, Sofia, Bulgaria; Department of Neurology, Medical University, Sofia, Bulgaria; Department of Cognitive Science and Psychology, New Bulgarian University, Sofia, Bulgaria.
  • Planté-Bordeneuve V; Department of Neurology- Amyloid Network-University Hospital Henri Mondor-Assistance Publique Hopitaux de Paris, IMRB INSERM U955 Team 10 "Biology of the Neuro-Muscular System", Créteil, France.
  • Chapman D; Pfizer, New York, New York, USA.
  • Amass L; Pfizer, New York, New York, USA.
JACC Heart Fail ; 9(10): 736-746, 2021 10.
Article em En | MEDLINE | ID: mdl-34391735
OBJECTIVES: Because patients with ATTRv cardiomyopathy are more likely to be male, this analysis aimed to increase information on associations between sex and genotype, phenotype, and degree of myocardial involvement in ATTRv amyloidosis. BACKGROUND: Transthyretin amyloid cardiomyopathy is a progressive, fatal disease that occurs due to accumulation of wild-type or variant (ATTRv) transthyretin amyloid fibrils in the myocardium. METHODS: The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing global longitudinal observational survey of patients with ATTR amyloidosis and asymptomatic carriers with TTR mutations. Data from THAOS (data cutoff: January 6, 2020) were analyzed to determine any sex-based differences in genotype, phenotype, and presence of cardiac and neurological symptoms in patients with ATTRv amyloidosis and in patients with ATTRv amyloidosis and cardiomyopathy. RESULTS: There were 2,790 patients with ATTRv amyloidosis enrolled in THAOS, with male patients more likely to have symptoms of cardiac involvement and a cardiac phenotype. Male prevalence was greater in patients with more severe cardiac manifestations of disease, as assessed with N-terminal pro-B-type natriuretic peptide, left-ventricular (LV) ejection fraction, mean LV wall thickness divided by height, and LV mass index divided by height. Sex, age at disease onset, and genotype category were identified by multivariate analyses as risk factors for the development of cardiomyopathy (defined as increased LV septum thickness divided by height). CONCLUSIONS: In this analysis, myocardial involvement was more frequent and pronounced in male patients with ATTRv amyloidosis, suggesting that there may be biological characteristics that inhibit myocardial amyloid infiltration in females or facilitate it in males.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neuropatias Amiloides Familiares / Insuficiência Cardíaca Tipo de estudo: Etiology_studies / Prognostic_studies / Risk_factors_studies Limite: Female / Humans / Male Idioma: En Revista: JACC Heart Fail Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Itália

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neuropatias Amiloides Familiares / Insuficiência Cardíaca Tipo de estudo: Etiology_studies / Prognostic_studies / Risk_factors_studies Limite: Female / Humans / Male Idioma: En Revista: JACC Heart Fail Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Itália