Introduction Hypertrophic cardiomyopathy (HCM) is an intrinsic myocardial disorder characterized by cardiac hypertrophy (wall thickness ≥ 15 mm), that is not explained by conditions of pressure overload (eg, hypertension, severe aortic stenosis). HCM is the most common genetic primary cardiomyopathy, with a prevalence estimated to be about one in 500 adults in the general population. More than 450 mutations have been identified in the 20 genes that cause different phenotypes. In most cases, HCM is associated with sarcomere protein gene […]

Introduction GCW, age 33, presents acute heart failure (HF), amenorrhea and darkening of the skin. Three months after this initial situation, the patient is referred to a large cardiology centre and is submitted to HF screening, and the suspicion of hemochromatosis is promptly raised. This disease affects the heart in only 15% of cases. It has been shown that early recognition and intervention may change the prognosis. This is a diagnosis that should be considered in all cases of HF […]

Introduction Graves’ disease (GD) is an autoimmune disease and the most common cause of thyrotoxicosis,, with multisystem involvement, that mainly affects women between 40 and 60 years of age. It is the most prevalent cause of autoimmune hyperthyroidism in pregnancy,, and it can be distinguished from gestational thyrotoxicosis due to the presence of diffuse goiter and previous history of hyperthyroidism. Clinical hyperthyroidism occurs in 0.2% of pregnant women. Heart failure (HF) is a rare manifestation of decompensated GD and it […]

Introduction Complete atrioventricular block (CAVB) is not an uncommon complication after surgical correction of tetralogy of Fallot (TOF). The choice of the ventricular pacing site in patients requiring pacemaker therapy depends on factors such as age, weight, presence of a venous anomaly, and intracardiac short -circuit. The harmful effects of ventricular pacing are most pronounced during right ventricular (RV) stimulation. Even then, RV pacing sites have been determined to be optimal in some patients with and without congenital heart disease. […]

Introduction Cardiac events are rarely associated with ophidian accidents, especially those involving snakes of the Elapidae family. We report on a snake bite victim who, within a few hours after the accident, developed myocardial infarction in the inferior wall with good clinical evolution. […]

Introduction Becker muscular dystrophy is a rare genetic disease with a birth incidence of approximately 1:30000 in males. The disease results from a X-linked recessive mutation on the gene located at Xp21, which encodes the dystrophin protein. Clinical manifestations include progressive skeletal muscle weakness and dilated cardiomyopathy which can lead to refractory heart failure. There is no correlation between the severity of cardiac manifestations and the genetic abnormalities. […]

Introduction Tachycardiomyopathy (TCM) is a rare, but potentially reversible cause of cardiomyopathy and cardiogenic shock. It is defined by a global left ventricular systolic dysfunction secondary to persistent tachyarrhythmia, with partial (in patients with previous structural disease), or total recovery (in patients without previous structural disease) after cardiac rhythm normalization. The most commonly implicated arrhythmia is atrial fibrillation. The hemodynamic changes that characterize the patient with TCM include: increase in ventricular telediastolic and telesystolic volumes, global hypokinesis, increase in ventricular […]

Introduction Familial Hypercholesterolemia (FH) is an autosomal dominant hereditary disease, a genetic disease of lipoprotein metabolism, mainly due to a defect in the LDLR gene encoding the LDL receptor.– The diagnosis is established by clinical and laboratory criteria and should always be a diagnostic hypothesis in patients with low-density lipoprotein cholesterol (LDLc) levels greater than 190 mg/dL; and can be confirmed by genetic tests that determine the mutation., Some diagnostic criteria have been proposed in an attempt to standardize and […]

Introduction Cardiac memory, also termed Chatterjee phenomenon, is a known yet uncommonly recognized entity in which myocardial repolarization is altered after abnormal ventricular activation, such as with artificial pacemakers, intermittent left bundle branch block, ventricular premature beats, ventricular pre-excitation or episodes of tachycardia. This phenomenon is characterized by persistent but reversible T-wave changes on surface electrocardiogram (ECG) induced by an abnormal electrical activation pattern. The extent and direction of T-wave deviation depend on the duration and direction of abnormal electrical […]

Introduction Aortic valve replacement (AVR) is an established treatment modality in patients with severe symptomatic aortic stenosis (AS)., Given the increased mortality and morbidity of AVR for the high-risk patients and the poor long-term results of balloon aortic valvuloplasty, transcatheter aortic valve implantation (TAVI) is increasingly accepted treatment option who are not candidates for AVR. We describe here a case of succesfully implanted Edwards SAPIEN XT transcatheter heart valve without aortography guidance due to multiple arterial access problems and complicated […]