Safety and Efficacy of Mavacamten and Aficamten in Patients With Hypertrophic Cardiomyopathy.

Background: Cardiac myosin inhibitors were recently developed to address the underlying pathophysiology of hypertrophic cardiomyopathy and to improve symptoms and quality of life. In this review, we evaluated the pharmacologic profile and clinical outcomes for mavacamten and aficamten, 2 cardiac myosin inhibitors investigated in symptomatic hypertrophic cardiomyopathy.

Methods And Results: Using a systematic search, 10 clinical trials with safety and efficacy data for either drug in obstructive hypertrophic cardiomyopathy (oHCM) and nonobstructive hypertrophic cardiomyopathy were included.

Left ventricular ejection fraction decline and cardiovascular events in suspected cardiomyopathy with excessive trabeculation: toward precision medicine.

Introduction And Objectives: Defining the probability of cardiomyopathy in individuals with excessive trabeculation of the left ventricle (ETLV) is an unmet clinical need. Our aims were: a) to describe the incidence and predictors of left ventricular ejection fraction (LVEF) decline and its correlation with major adverse cardiovascular events (MACE); and b) to identify prognostic factors in low-risk individuals.

Methods: Retrospective multicenter study in patients with ETLV and suspected cardiomyopathy.

Key priorities for the implementation of the 2023 ESC Guidelines for the Management of Cardiomyopathies in low resource settings.

ESC Guidelines provide best practice, evidence-based recommendations for diagnosing and treating patients with cardiovascular diseases. It is not always possible for best practices to be followed, however, particularly in low-resource settings. To address this issue, a set of guideline-related documents were created to identify key priorities for users in these settings.

Electrophysiological Phenotype-Genotype Study of Sustained Monomorphic Ventricular Tachycardia in Inherited, High Arrhythmic Risk, Left Ventricular Cardiomyopathy.

Background: Among inherited cardiomyopathies involving the left ventricle, whether dilated or not, certain genotypes carry a well-established arrhythmic risk, notably manifested as sustained monomorphic ventricular tachycardia (SMVT). Nonetheless, the precise localization and electrophysiological profile of this substrate remain undisclosed across different genotypes.

Methods: Patients diagnosed with cardiomyopathy and left ventricle involvement due to high-risk genetic variants and SMVT treated by electrophysiological study were recruited from 18 European/US centers.