RESUMO
Takotsubo syndrome (TTS) is a rare disease mimicking acute coronary syndrome, often triggered by physical or emotional stress, and characterized by transient left ventricular dysfunction. Recurrences are described in about 5% of cases and may have different clinical and imaging patterns. In the present report, SARS-COV-2 infection, even in the absence of symptoms and overt emotional stress, seems correlated with recurrence of TTS, due to the absence of other recognized triggers. The hypothesis is that in predisposed patients, events like catecholamine-induced myocyte injury, direct viral damage, cytokine storm, immune-mediated damage, and procoagulant state, all possibly induced by the infection, may elicit endothelial dysfunction as substrate for TTS onset.
RESUMO
AIMS: Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis. We aimed to examine the development of a regional network for the diagnosis and management of ATTR-CA and describe a cohort of patients with ATTR-CA, investigate diagnostic pathways and assess clinical outcomes according to diagnosis periods. METHODS: We performed a survey study analyzing answers from 11 cardiology centers and we conducted a retrospective study including patients with ATTR-CA attending a referral center between 1 January 2012 and 31 December 2022, and categorized by the period of diagnosis (2012-2016 and 2017-2022). RESULTS: Over the years, a growing number of patients reached a diagnosis and were treated in the surveyed nonreferral centers of the region. The retrospective study showed a more significant diagnostic delay in the earlier period rather than the later one [13.4 (5-30.2) vs. 10.6 (5.0-17.9) months, P = 0.04]. Patients diagnosed after 2017 showed a greater survival rate than those diagnosed earlier ( P = 0.02). In the multivariate analysis, the year of diagnosis from 2017 remained independently associated with mortality [hazard ratio (HR) 0.46, 95% confidence interval (CI) 0.28-0.79; P = 0.005]. CONCLUSION: This study emphasized the shift toward noninvasive diagnostic criteria. It revealed a positive impact on patient survival and disease management with the use of disease-modifying therapies and diagnostic developments in more recent years. The findings underscore the importance of disease awareness and networking to reduce diagnostic delays and enhance patient journeys for ATTR-CA.
Assuntos
Neuropatias Amiloides Familiares , Cardiomiopatias , Diagnóstico Tardio , Encaminhamento e Consulta , Humanos , Estudos Retrospectivos , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/terapia , Neuropatias Amiloides Familiares/mortalidade , Masculino , Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Feminino , Idoso , Encaminhamento e Consulta/estatística & dados numéricos , Fatores de Tempo , Itália , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Pesquisas sobre Atenção à Saúde , Tempo para o Tratamento , Valor Preditivo dos Testes , Procedimentos ClínicosRESUMO
AIM: Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival. METHODS AND RESULTS: This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different 'pathways' according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III-IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III-IV and some comorbidities but not the HF pathway were independently associated with worse survival. CONCLUSIONS: Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.
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Neuropatias Amiloides Familiares , Cardiomiopatias , Insuficiência Cardíaca , Humanos , Pré-Albumina/genética , Pré-Albumina/metabolismo , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/epidemiologia , Neuropatias Amiloides Familiares/complicações , Estudos Retrospectivos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/complicações , Cardiomiopatias/diagnóstico , Cardiomiopatias/epidemiologia , Cardiomiopatias/complicaçõesRESUMO
Cardiomyopathies are a heterogeneous group of cardiac diseases for which diagnosis and treatment are not always simple. The diagnosis of cardiomyopathy, in particular the etiology, comes from an integration between symptoms and results collected by several instrumental exams. The brain storming for the diagnosis includes also the identification of the "red flags", i.e. the pathognomonic features for each etiology that can drive the choice of appropriate diagnostic tests and therapy. In this review, we provide a step by step approach in order to help cardiologists, not specifically dedicated to cardiomyopathies, to draw the diagnosis, therapy and follow-up. This approach will be accompanied by the consultation of other specialists to discuss together the results of the exams performed and to deepen extracardiac signs and symptoms.
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Cardiomiopatias/diagnóstico , Cardiomiopatias/genética , Fenótipo , Avaliação de Sintomas , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/genética , Displasia Arritmogênica Ventricular Direita/terapia , Cardiomiopatias/terapia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/terapia , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Humanos , Imagem Cinética por Ressonância Magnética , Tomografia por Emissão de Pósitrons , Encaminhamento e Consulta , Sarcoidose/diagnósticoAssuntos
Desfibriladores Implantáveis/efeitos adversos , Soluço/etiologia , Marca-Passo Artificial/efeitos adversos , Distúrbios da Voz/etiologia , Músculos Abdominais/fisiopatologia , Idoso , Demência/complicações , Eletrodos , Falha de Equipamento , Feminino , Soluço/diagnóstico , Humanos , Obesidade/complicações , Músculos Peitorais/fisiopatologia , Radiografia Torácica , Reoperação , Fatores de Risco , Síndrome , Distúrbios da Voz/diagnósticoRESUMO
Coronary care units (CCUs) should ensure the best intensive therapy for all critical cardiologic patients and not only for patients with acute coronary heart disease. Such structures apply the Hub & Spoke model, which consists of an integrated network of services allowing a health organization in which different realities interact and collaborate; this organization is composed of referral core centers (Hubs) and smaller structures (Spokes) referring to Hubs that are engaged in selection, channeling of patients in the acute phase, and for follow-up care of patients in the post-acute phase. The CCUs, based on the organizational reality in which they operate, must hospitalize and dismiss complex patients in a brief lapse of time. Criteria for CCU admission and length of stay are still ill-defined. Therefore, the following paper, summarizing the contents of the recent CCU convention at the ANMCO congress, attempts to define the priorities for hospitalization in the CCU, based on three different levels of evidence: level A indication (immediate mandatory admission); level B indication (immediate admission, the availability of beds allowing); level C indication (admission not indicated, but possible in the absence of other alternatives, e.g. limited bed availability in other intensive care units). Concerning the duration of stay within the CCU, clear-cut indications are difficult, but the concept is emphasized that the length of stay should be minimized, given the limited bed availability, in order to ensure the availability of intensive monitoring to all critical patients.