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Cardiac amyloidosis (CA) is a form of cardiomyopathy characterized by the extracellular deposit of protein fibers in the myocardium, leading to the development of heart failure, arrhythmias, and electrical conduction system alterations. It is known that most cardiomyopathies have a close relationship with heart rhythm abnormalities, however, CA is specially related to different kinds of arrhythmias even in pre-diagnosis stages. Arrhythmias like atrial fibrillation are present in up to 70% of patients with CA associated with a high risk of cardioembolic complications independent of the risk stratification. Ventricular arrhythmias are frequent, but the use of implantable cardioverter defibrillator has not been demonstrated to improve survival. The Atrial-Ventricular node disease is also common, and is frequently associated with the implantation of a pacemaker, even in asymptomatic patients. In this review, we clarify the recommendations of the most current guidelines, summarize historical and contemporaneous data and describe evidence-based strategies for the management of arrhythmias and their complications in CA., Competing Interests: Conflicto de interés: Los autores declaran no tener conflictos de interés

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon inherited myocardial disorder characterised by fibro-fatty inflammation affecting the right and left ventricles. It most commonly presents with palpitations or syncope but sudden death may occur, especially in young males. Methods: Diagnosis is not possible with a single test and may be difficult. Task Force criteria agreed in 1994 comprise major and minor criteria spanning structural abnormalities, ECG appearances, arrhythmias, family history of premature death and myocardial histology. Modified criteria were introduced in 2010 to improve sensitivity. Results: Arrhythmogenic right ventricular cardiomyopathy is a desmosomal disease. Mutations have been detected in five desmosomal genes, most frequently in plakophilin-2 (PKP2) and multiple mutations are also reported. Antiarrhythmic drugs such as sotalol and amiodarone may improve symptoms but are unproven to increase survival. An implantable defibrillator is appropriate in individuals surviving cardiac arrest or sustained ventricular tachycardia, but there is not yet consensus about prophylactic treatment of Task Force positive but asymptomatic individuals. Conclusions: Arrhythmogenic right ventricular cardiomyopathy is more common than previously believed. Preliminary evidence supports improved sensitivity without loss of specificity using the revised Task Force criteria. The genetics of the disease are complex but should ultimately advance diagnosis and management. [ABSTRACT FROM AUTHOR]

Background: Myotonic dystrophy type 2 (DM2) is a genetic disorder characterized by skeletal muscle symptoms, metabolic changes, and cardiac involvement. Histopathologic alterations of the skeletal muscle include fibrosis and fatty infiltration. The aim of this study was to investigate whether subclinical cardiac involvement in DM2 is already detectable in preserved left ventricular function by cardiovascular magnetic resonance., Methods and Results: Twenty-seven patients (mean age, 54±10 years; 20 females) with a genetically confirmed diagnosis of DM2 were compared with 17 healthy age- and sex-matched controls using a 1.5 T magnetic resonance imaging. For myocardial tissue differentiation, T1 and T2 mapping, fat/water-separated imaging, focal fibrosis imaging (late gadolinium enhancement [LGE]), and (1)H magnetic resonance spectroscopy were performed. Extracellular volume fraction was calculated. Conduction abnormalities were diagnosed based on Groh criteria. LGE located subepicardial basal inferolateral was detectable in 22% of the patients. Extracellular volume was increased in this region and in the adjacent medial inferolateral segment (P=0.03 compared with healthy controls). In 21% of patients with DM2, fat deposits were detectable (all women). The control group showed no abnormalities. Myocardial triglycerides were not different in LGE-positive and LGE-negative subjects (P=0.47). Six patients had indicators for conduction disease (60% of LGE-positive patients and 12.5% of LGE-negative patients)., Conclusions: In DM2, subclinical myocardial injury was already detectable in preserved left ventricular ejection fraction. Extracellular volume was also increased in regions with no focal fibrosis. Myocardial fibrosis was related to conduction abnormalities., (© 2016 American Heart Association, Inc.)