RESUMO
BACKGROUND: Although transthyretin cardiac amyloidosis (ATTR-CA) is often underdiagnosed, clinical suspicion is essential for early diagnosis. OBJECTIVES: The aim of this study was to develop and validate a feasible prediction model and score to facilitate the diagnosis of ATTR-CA. METHODS: This retrospective multicenter study enrolled consecutive patients who underwent 99mTc-DPD scintigraphy for suspected ATTR-CA. ATTR-CA was diagnosed if Grade 2 or 3 cardiac uptake was evidenced on 99mTc-DPD scintigraphy in the absence of a detectable monoclonal component or by demonstration of amyloid by biopsy. A prediction model for ATTR-CA diagnosis was developed in a derivation sample of 227 patients from 2 centers using multivariable logistic regression with clinical, electrocardiography, analytical, and transthoracic echocardiography variables. A simplified score was also created. Both of them were validated in an external cohort (n = 895) from 11 centers. RESULTS: The obtained prediction model combined age, gender, carpal tunnel syndrome, interventricular septum in diastole thickness, and low QRS interval voltages, with an area under the curve (AUC) of 0.92. The score had an AUC of 0.86. Both the T-Amylo prediction model and the score showed a good performance in the validation sample (ie, AUC: 0.84 and 0.82, respectively). They were tested in 3 clinical scenarios of the validation cohort: 1) hypertensive cardiomyopathy (n = 327); 2) severe aortic stenosis (n = 105); and 3) heart failure with preserved ejection fraction (n = 604), all with good diagnostic accuracy. CONCLUSIONS: The T-Amylo is a simple prediction model that improves the prediction of ATTR-CA diagnosis in patients with suspected ATTR-CA.
Assuntos
Neuropatias Amiloides Familiares , Cardiomiopatias , Humanos , Pré-Albumina , Neuropatias Amiloides Familiares/diagnóstico por imagem , Valor Preditivo dos Testes , CoraçãoRESUMO
Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.
Assuntos
Transplante de Coração , Escleroderma Sistêmico , Humanos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/cirurgiaRESUMO
Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.(AU)
El compromiso cardíaco en la esclerosis sistémica (ES) es raro, pero conlleva un mal pronóstico a corto plazo. La evidencia relativa al trasplante cardíaco (TC) es escasa y se basa en la experiencia con los casos aislados. Presentamos este caso con el objetivo de analizar las características de un paciente con esclerosis sistémica sometido a un trasplante exitoso.(AU)
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico , Transplante de Coração , Miosite , Assistência ao Paciente , ReumatologiaRESUMO
Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.
