Clinical characteristics of wild-type transthyretin cardiac amyloidosis – Disproving myths.

Repositorio Dspace/Manakin

Clinical characteristics of wild-type transthyretin cardiac amyloidosis – Disproving myths.

Mostrar el registro completo del ítem

Título: Clinical characteristics of wild-type transthyretin cardiac amyloidosis – Disproving myths.
Autor: González López, Esther; Gagliardi, Christian; Domínguez, Fernando; Quarta, Cristina Candida; De Haro del Moral, Francisco Javier; Milandri, Agnese; Salas, Clara; Cinelli, Mario; Cobo Marcos, Marta; Lorenzini, Massimiliano; Lara Pezzi, Enrique; Foffi, Serena; Alonso Pulpón, Luis; Rapezzi, Claudio; García Pavía, Pablo
Resumen: Aims: Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterised by concentric LV hypertrophy, preserved LVEF and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Methods and Results: Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy 12mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion). Mutations in TTR were excluded in all cases. The study cohort comprised 108 patients (78.68 years); 67 (62%) diagnosed invasively and 41 (38%) non-invasively. Twenty patients (19%) were females. An asymmetric hypertrophy pattern was observed in 25 (23%) patients. Mean LVEF was 5214%, with 39 patients (37%) showing a LVEF<50%. Atrial fibrillation (56%) and a pseudo-infarct pattern (63%) were the commonest ECG findings. Only 22 patients fulfilled QRS low-voltage criteria while 10 showed LV hypertrophy on ECG. Although heart failure was the most frequent profile leading to diagnosis (68%), 7% of individuals presented with atrioventricular block and 11% were diagnosed incidentally. Almost one third (35; 32%) were previously misdiagnosed. Conclusion: The clinical spectrum of ATTRwt is heterogeneous and differs from the classic phenotype: women are affected in a significant proportion; asymmetric LV hypertrophy and impaired LVEF are not rare and only a minority have low QRS voltages. Clinicians should be aware of the broad clinical spectrum of ATTRwt to correctly identify an entity for which a number of disease-modifying treatments are under investigation.
Identificador universal:
Fecha: 2017

Ficheros en el ítem

Ficheros Tamaño Formato Ver
Clinical characteristics pablo pavia.pdf 832.7Kb PDF Ver/Abrir

Este ítem aparece en la(s) siguiente(s) colección(ones)

Mostrar el registro completo del ítem

Atribución-NoComercial-SinDerivadas 3.0 España Excepto si se señala otra cosa, la licencia del ítem se describe comoAtribución-NoComercial-SinDerivadas 3.0 España

Buscar en DSpace

Búsqueda avanzada


Mi cuenta