Publicação
Portuguese recommendations on transthyretin amyloid cardiomyopathy: a step toward disease awareness, prompt referral and early diagnosis and treatment
| datacite.subject.sdg | 03:Saúde de Qualidade | |
| datacite.subject.sdg | 10:Reduzir as Desigualdades | |
| datacite.subject.sdg | 04:Educação de Qualidade | |
| dc.contributor.author | Marques, Nuno | |
| dc.contributor.author | Azevedo, Olga | |
| dc.date.accessioned | 2026-04-30T11:23:27Z | |
| dc.date.available | 2026-04-30T11:23:27Z | |
| dc.date.issued | 2025-02 | |
| dc.description.abstract | Transthyretin amyloidosis (ATTR) is caused by the extracellular deposition of amyloid fibrils of wild-type (ATTRwt) or variant (ATTRv) transthyretin (TTR). While ATTRwt amyloidosis is essentially a cardiac disease, ATTRv amyloidosis may present with different phenotypes, ranging from predominantly cardiac to predominantly neurologic, or even mixed phenotypes, depending on the TTR gene variant.1---3 Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive cardiomyopathy that causes heart failure, dysrhythmias and conduction block, which eventually lead to death.1 Median survival following diagnosis in untreated patients with ATTR-CM is 3.6---4.8 years in the wild-type form, 2.6 years in ATTR-CM due to the Val142Ile variant and 5.8 years in ATTR-CM caused by other TTR variants.4,5 To improve the prognosis of ATTR-CM, it is important to implement strategic measures that (i) increase awareness of ATTR amyloidosis, (ii) ensure early referral of cases with suspicion of ATTR-CM, (iii) promote early screening, diagnosis and treatment of ATTR-CM, and (iv) assure appropriate symptomatic management of the disease. However, some questions remain concerning who should be screened for ATTR-CM, and international recommendations differ regarding the red flags that should prompt screening for ATTR-CM.6 | eng |
| dc.identifier.doi | 10.1016/j.repc.2024.11.010 | |
| dc.identifier.issn | 0870-2551 | |
| dc.identifier.uri | http://hdl.handle.net/10400.1/28825 | |
| dc.language.iso | eng | |
| dc.peerreviewed | yes | |
| dc.publisher | Elsevier | |
| dc.relation.ispartof | Revista Portuguesa de Cardiologia | |
| dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/4.0/ | |
| dc.title | Portuguese recommendations on transthyretin amyloid cardiomyopathy: a step toward disease awareness, prompt referral and early diagnosis and treatment | eng |
| dc.title.alternative | Recomendações portuguesas sobre ATTR-CM: um passo para a sensibilização da doença para a referenciação rápida e para o diagnóstico precoce e tratamento | por |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| oaire.citation.endPage | 5 | |
| oaire.citation.startPage | 3 | |
| oaire.citation.title | Revista Portuguesa de Cardiologia | |
| oaire.citation.volume | 44 | |
| oaire.version | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |
| person.familyName | Marques | |
| person.givenName | Nuno | |
| person.identifier.orcid | 0000-0003-0275-2807 | |
| relation.isAuthorOfPublication | 1b66ba1f-d295-4211-b41e-c0f0b622eea0 | |
| relation.isAuthorOfPublication.latestForDiscovery | 1b66ba1f-d295-4211-b41e-c0f0b622eea0 |