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Breakthrough advances enhancing care in ATTR amyloid cardiomyopathy.
Porcari, Aldostefano; Sinagra, Gianfranco; Gillmore, Julian D; Fontana, Marianna; Hawkins, Philip N.
Afiliación
  • Porcari A; National Amyloidosis Centre, Division of Medicine, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK; Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Triest
  • Sinagra G; Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste 34149, Italy; European Reference Network for rare, low prevalence and complex diseases of the heart (ERN GUARD-Heart), Italy.
  • Gillmore JD; National Amyloidosis Centre, Division of Medicine, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK.
  • Fontana M; National Amyloidosis Centre, Division of Medicine, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK.
  • Hawkins PN; National Amyloidosis Centre, Division of Medicine, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK.
Eur J Intern Med ; 123: 29-36, 2024 May.
Article en En | MEDLINE | ID: mdl-38184468
ABSTRACT
Transthyretin amyloid cardiomyopathy (ATTR-CM) has been traditionally considered a rare and inexorably fatal condition. ATTR-CM now is an increasingly recognized cause of heart failure (HF) and mortality worldwide with effective pharmacological treatments. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have transformed the diagnosis of ATTR-CM, which is now possible without recourse to endomyocardial biopsy in ≈70 % of cases. Many patients are now diagnosed at an earlier stage. Echocardiography and cardiac magnetic resonance have enabled identification of patients with possible ATTR-CM and more accurate prognostic stratification. Although radionuclide scintigraphy with 'bone' tracers has an established diagnostic value, the diagnostic performance of the bone tracers validated for non-invasive confirmation of ATTR-CM may not be equal. Characterising the wider clinical phenotype of patients with ATTR-CM has enabled identification of features with potential for earlier diagnosis such as carpal tunnel syndrome. Therapies able to slow or halt ATTR-CM progression and increase survival are now available and there is also evidence that patients may benefit from specific conventional HF medications. Cutting-edge research in the field of antibody-mediated removal of ATTR deposits compellingly suggest that ATTR-CM is a truly reversible disorder, bringing hope for patients even with advanced disease. A wide horizon of possibilities is unfolding and awaits discovery.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Neuropatías Amiloides Familiares / Insuficiencia Cardíaca / Cardiomiopatías Límite: Humans Idioma: En Revista: Eur J Intern Med Asunto de la revista: MEDICINA INTERNA Año: 2024 Tipo del documento: Article Pais de publicación: Países Bajos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Neuropatías Amiloides Familiares / Insuficiencia Cardíaca / Cardiomiopatías Límite: Humans Idioma: En Revista: Eur J Intern Med Asunto de la revista: MEDICINA INTERNA Año: 2024 Tipo del documento: Article Pais de publicación: Países Bajos