Emilio Blanco-López , Jorge Martínez-del Río , Alba López-Calles , Martín Negreira-Caamaño , Daniel Águila-Gordo , Pablo Soto-Martín , Maria Maeve Soto-Pérez , Andrez Felipe Cubides-Novoa , Maria Gonzalez-Barderas , Ignacio Sánchez-Pérez , Jesús Piqueras-Flores
{"title":"心脏淀粉样变性和红旗:自然史及其对死亡率的影响。","authors":"Emilio Blanco-López , Jorge Martínez-del Río , Alba López-Calles , Martín Negreira-Caamaño , Daniel Águila-Gordo , Pablo Soto-Martín , Maria Maeve Soto-Pérez , Andrez Felipe Cubides-Novoa , Maria Gonzalez-Barderas , Ignacio Sánchez-Pérez , Jesús Piqueras-Flores","doi":"10.1016/j.medcli.2024.08.006","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction and objectives</h3><div>Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence.</div></div><div><h3>Patients and methods</h3><div>Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them.</div></div><div><h3>Results</h3><div>102 patients (81.6<!--> <!-->±<!--> <!-->7.7 years; 84,3% males) with a diagnosis of CA (89.2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8.4<!--> <!-->±<!--> <!-->2.3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥<!--> <!-->10 RFs being associated with higher mortality.</div></div><div><h3>Conclusions</h3><div>RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.</div></div>","PeriodicalId":18578,"journal":{"name":"Medicina Clinica","volume":"164 2","pages":"Pages 61-68"},"PeriodicalIF":2.6000,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Amiloidosis cardiaca y red flags: historia natural e impacto en la morbimortalidad\",\"authors\":\"Emilio Blanco-López , Jorge Martínez-del Río , Alba López-Calles , Martín Negreira-Caamaño , Daniel Águila-Gordo , Pablo Soto-Martín , Maria Maeve Soto-Pérez , Andrez Felipe Cubides-Novoa , Maria Gonzalez-Barderas , Ignacio Sánchez-Pérez , Jesús Piqueras-Flores\",\"doi\":\"10.1016/j.medcli.2024.08.006\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Introduction and objectives</h3><div>Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence.</div></div><div><h3>Patients and methods</h3><div>Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them.</div></div><div><h3>Results</h3><div>102 patients (81.6<!--> <!-->±<!--> <!-->7.7 years; 84,3% males) with a diagnosis of CA (89.2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8.4<!--> <!-->±<!--> <!-->2.3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥<!--> <!-->10 RFs being associated with higher mortality.</div></div><div><h3>Conclusions</h3><div>RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.</div></div>\",\"PeriodicalId\":18578,\"journal\":{\"name\":\"Medicina Clinica\",\"volume\":\"164 2\",\"pages\":\"Pages 61-68\"},\"PeriodicalIF\":2.6000,\"publicationDate\":\"2025-01-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Medicina Clinica\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0025775324005591\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Medicina Clinica","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0025775324005591","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
Amiloidosis cardiaca y red flags: historia natural e impacto en la morbimortalidad
Introduction and objectives
Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence.
Patients and methods
Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them.
Results
102 patients (81.6 ± 7.7 years; 84,3% males) with a diagnosis of CA (89.2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8.4 ± 2.3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥ 10 RFs being associated with higher mortality.
Conclusions
RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.
期刊介绍:
Medicina Clínica, fundada en 1943, es una publicación quincenal dedicada a la promoción de la investigación y de la práctica clínica entre los especialistas de la medicina interna, así como otras especialidades. Son características fundamentales de esta publicación el rigor científico y metodológico de sus artículos, la actualidad de los temas y, sobre todo, su sentido práctico, buscando siempre que la información sea de la mayor utilidad en la práctica clínica.