Background: Rare genetic variants in TNNI3K encoding troponin-I interacting kinase have been linked to a distinct syndrome consisting primarily of supraventricular tachycardias and variably expressed conduction disturbance and dilated cardiomyopathy in 2 families.
Objective: The purpose of this study was to identify new genetic variants associated with inherited supraventricular tachycardias, cardiac conduction disease, and cardiomyopathy.
Methods: We conducted next generation sequencing in 3 independent multigenerational families with atrial/junctional tachycardia with or without conduction disturbance, dilated cardiomyopathy, and sudden death.
Background: Brugada syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) that seldom manifests or is recognized in childhood.
Objectives: The objectives of this study were to describe the clinical presentation of pediatric BrS to identify prognostic factors for risk stratification and to propose a data-based approach management.
Methods: We studied 106 patients younger than 19 years at diagnosis of BrS enrolled from 16 European hospitals.
Aims: In low-flow, low-gradient aortic stenosis (LF/LG AS), the assessment of contractile reserve (CR) by dobutamine stress echocardiography (DSE) is recommended for risk stratification and treatment strategy. However, DSE may show limitations in cases of left ventricular dyssynchrony (LVD). The impact of LVD in LF/LG AS, and the feasibility of CRT in this setting have never been evaluated.
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