التفاصيل البيبلوغرافية
| العنوان: |
Clinical characteristics of wild-type transthyretin cardiac amyloidosis – Disproving myths. |
| المؤلفون: |
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 |
| بيانات النشر: |
European heart journal |
| سنة النشر: |
2017 |
| المجموعة: |
Universidad Francisco de Vitoria: Depósito Digital FV (DDFV) |
| مصطلحات موضوعية: |
Amiloidosis cardiaca, Transtiretina, Gammagrafía Tc-DPD |
| الوصف: |
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 (78.68 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 5214%, 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. ; pre-print ; 833 KB |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| تدمد: |
1522-9645 |
| العلاقة: |
http://hdl.handle.net/10641/1295Test |
| الإتاحة: |
http://hdl.handle.net/10641/1295Test |
| حقوق: |
Atribución-NoComercial-SinDerivadas 3.0 España ; http://creativecommons.org/licenses/by-nc-nd/3.0/esTest/ ; openAccess |
| رقم الانضمام: |
edsbas.BCCC8498 |
| قاعدة البيانات: |
BASE |
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