Abstract Background Systemic amyloidosis is a disease with heterogeneous clinical manifestations. Diagnosis depends on clinical suspicion combined with specific complementary methods. Objective To describe the clinical, laboratory, electrocardiographic, and imaging profile in patients with systemic amyloidosis with cardiac involvement. Methods This study was conducted with a convenience sample, analyzing clinical, laboratory, electrocardiographic, echocardiographic, nuclear medicine, and magnetic resonance data. Statistical significance was set at p < 0.05. Results A total of 105 patients were evaluated (median age of 66 years), [...]

Abstract Background: Diffuse cardiac fibrosis is an important factor in the prognostic assessment of patients with ventricular dysfunction. Cardiovascular magnetic resonance imaging (CMR) native T1 mapping is highly sensitive and considered an independent predictor of all-cause mortality and heart failure (HF) development in patients with cardiomyopathy. Objectives: To evaluate the feasibility of native T1 mapping assessment in patients with HF in a cardiology referral hospital and its association with structural parameters and functional profile. Methods: Cross-sectional study with adult patients […]

Introduction Isolated right ventricular myocardial infarction is extremely rare, and it is often silent, with only 25% of patients developing clinically evident hemodynamic manifestations on presentation. Current management of acute myocardial infarction is based on prompt diagnosis and immediate revascularization. About 90% of patients presenting with ST-segment elevation myocardial infarction have an explanatory coronary artery stenosis or occlusion. Myocardial infarction with non-obstructive coronary arteries (MINOCA) should lead the treating physician to investigate underlying causes, since failure to identify the underlying […]

Introduction Three-dimensional echocardiography (3DE) plays an increasingly important role in the diagnosis of valvular heart disease, in the assessment of valvular morphology in an anatomical manner, and in establishing valve repairability, beyond the limitations of conventional two-dimensional echocardiography (2DE). We report the case of a young patient presenting with acute anterior ST-segment elevation myocardial infarction and severe mitral regurgitation (MR) after successful primary percutaneous coronary intervention (PCI) of the left anterior descending artery, whose three-dimensional transesophageal echocardiography (3D TEE) revealed […]

Clinical data A 65-year-old female patient was hospitalized, initially due to an episode of melena. Anticoagulation was suspended, and upper gastrointestinal endoscopy was performed, without detecting bleeding. In 2005, she had late post-operative history of mechanical aortic prosthesis and Bentall and de Bono surgery due to Stanford A aortic dissection; she was a former tobacco user, with obesity and chronic atrial fibrillation under regular use of warfarin. The following day, she had an acute myocardial infarction with ST-segment elevation in […]

Introduction Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive disease characterized by the formation of xanthomatous lesions in many tissues, particularly the brain and tendons. The disorder is a consequence of the reduced production of bile acids, predominantly chenodeoxycholic acid (CDCA), and an increased formation of cholestanol. Common clinical manifestations include infant-onset diarrhea and juvenile-onset bilateral cataract, usually followed by tendon xanthomas and progressive neurological dysfunction. The final diagnosis is based on biochemical abnormalities, including elevated plasma cholestanol level and increased […]