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Sex differences among patients with transthyretin amyloid cardiomyopathy - from diagnosis to prognosis.
Patel, Rishi K; Ioannou, Adam; Razvi, Yousuf; Chacko, Liza; Venneri, Lucia; Bandera, Francesco; Knight, Daniel; Kotecha, Tushar; Martinez-Naharro, Ana; Masi, Ambra; Porcari, Aldostefano; Brown, James; Patel, Kiara; Manisty, Charlotte; Moon, James; Rowczenio, Dorota; Gilbertson, Janet A; Sinagra, Gianfranco; Lachmann, Helen; Wechalekar, Ashutosh; Petrie, Aviva; Whelan, Carol; Hawkins, Philip N; Gillmore, Julian D; Fontana, Marianna.
Afiliación
  • Patel RK; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Ioannou A; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Razvi Y; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Chacko L; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Venneri L; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Bandera F; Heart Failure Unit, Cardiology University Department, IRCCS Policlinico San Donato, Milan, Italy.
  • Knight D; Department for Biomedical Sciences for Health, University of Milano, Milan, Italy.
  • Kotecha T; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Martinez-Naharro A; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Masi A; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Porcari A; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Brown J; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Patel K; Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.
  • Manisty C; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Moon J; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Rowczenio D; Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit, and the Inherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, London, UK.
  • Gilbertson JA; Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit, and the Inherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, London, UK.
  • Sinagra G; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Lachmann H; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Wechalekar A; Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.
  • Petrie A; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Whelan C; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Hawkins PN; Eastman Dental Institute, University College London, London, UK.
  • Gillmore JD; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
  • Fontana M; National Amyloidosis Centre, Division of Medicine, University College London, London, UK.
Eur J Heart Fail ; 24(12): 2355-2363, 2022 12.
Article en En | MEDLINE | ID: mdl-36575133
AIMS: Transthyretin amyloid cardiomyopathy (ATTR-CM) is predominantly diagnosed in men. The few available studies suggest affected women have a more favourable cardiac phenotype. We aimed to characterize sex differences among consecutive patients with non-hereditary and two prevalent forms of hereditary (h)ATTR-CM diagnosed over a 20-year period. METHODS AND RESULTS: Analysis of deep phenotyping at presentation, changes on serial echocardiography and overall prognosis were evaluated. In total, 1732 consecutive patients were studied, comprising: 1095 with wild-type (wt)ATTR-CM; 206 with T60A-hATTR-CM; and 431 with V122I-hATTR-CM. Female prevalence was greater in T60A-hATTR-CM (29.6%) and V122I-hATTR-CM (27.8%) compared to wtATTR-CM (6%). At presentation, females were 3.3 years older than males (wtATTR-CM: 81.9 vs. 77.8 years; T60A-hATTR-CM: 68.7 vs. 65.1 years; V122I-hATTR-CM: 77.1 vs. 74.9 years). Body size significantly influenced measures of disease severity; when indexed, overall structural and functional phenotype was similar between sexes, the few significant differences suggested a mildly worse phenotype in females. No significant differences were observed in both disease progression on serial echocardiography and mortality across the overall population (p = 0.459) and when divided by genotype (wtATTR-CM: p = 0.730; T60A-hATTR-CM: p = 0.161; V122I-hATTR-CM: p = 0.056). CONCLUSION: This study of a well-characterized large cohort of ATTR-CM patients did not demonstrate overall differences between sexes in either clinical phenotype, when indexed, or with respect to disease progression and prognosis. Non-indexed wall thickness measurements may have contributed to both under-representation and delays in diagnosis for affected females and highlights the potential role of utilizing indexed echocardiographic parameters for a more accurate assessment of patients at diagnosis and for disease prognostication.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Neuropatías Amiloides Familiares / Insuficiencia Cardíaca / Cardiomiopatías Tipo de estudio: Diagnostic_studies / Prognostic_studies / Risk_factors_studies Límite: Female / Humans / Male Idioma: En Revista: Eur J Heart Fail Asunto de la revista: CARDIOLOGIA Año: 2022 Tipo del documento: Article Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Neuropatías Amiloides Familiares / Insuficiencia Cardíaca / Cardiomiopatías Tipo de estudio: Diagnostic_studies / Prognostic_studies / Risk_factors_studies Límite: Female / Humans / Male Idioma: En Revista: Eur J Heart Fail Asunto de la revista: CARDIOLOGIA Año: 2022 Tipo del documento: Article Pais de publicación: Reino Unido