Arrhythmic genotypes in dilated cardiomyopathy and risk of advanced heart failure.

Background And Aims: Certain genetic forms of dilated cardiomyopathy (DCM) entail a higher arrhythmic risk. It is unknown whether DCM patients with high-risk arrhythmic genotypes also develop more advanced heart failure (AHF) complications. AHF events were studied according to DCM genotype.

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.

Vector field heterogeneity as a novel omnipolar mapping metric for functional substrate characterization in scar-related ventricular tachycardias.

Background: Vector field heterogeneity (VFH) is a novel omnipolar metric to quantify local propagation heterogeneities that may identify functionally critical sites for ablation in scar-related ventricular tachycardia (VT).

Objective: This study aims to assess the diagnostic value of VFH to identify abnormal propagation patterns during ventricular substrate mapping and compare VFH in VT isthmus sites (IS), low-voltage bystander area (LVA) , and normal voltage areas (NVAa).

Methods: Substrate maps acquired with a 16-pole grid catheter in patients with scar-related VT were segmented into sites corresponding to IS, LVA, and NVA (defined as omnipolar voltages > and <1.

Comparison of automated subcutaneous defibrillator screening between different pacing sites in cardiac pacing device carriers.

Aims: The compatibility of cardiac pacing with the presence of a subcutaneous implantable cardioverter-defibrillator (S-ICD) has been investigated, but S-ICD screening test results have not been compared among different pacing sites. The objective was to compare S-ICD screening results among different cardiac pacing sites and to assess the electrocardiographic predictors of success.

Methods And Results: This prospective single-centre study conducted automated S-ICD screening in 102 carriers of cardiac pacing devices in conduction system (CSP), biventricular (BVP), right ventricular outflow tract (RVOT), or right ventricular apex (RVA) pacing sites.

Septal fascicle involvement during a Purkinje-related ventricular tachycardia: Electroanatomic correlation using omnipolar technology.

Idiopathic verapamil-sensitive fascicular ventricular tachycardia (VT) is the most common form of Purkinje-related ventricular tachycardia (PRVT). Left septal fascicle (LSF) involvement and its connections with the other fascicles, have been recently reported as a pathophysiologic mechanism for this form of PRVT. We describe a case of idiopathic PRVT with LSF involvement using omnipolar technology (OT) mapping in relation to a false tendon.

Lifestyle physical activity and rapid-rate non-sustained ventricular tachycardia in arrhythmogenic cardiomyopathy.

Objective: To investigate the association of accelerometer-measured lifestyle physical activity with rapid-rate non-sustained ventricular tachycardias (RR-NSVTs) in patients with arrhythmogenic cardiomyopathy (AC).

Methods: This multicentre, observational study enrolled 72 patients with AC, including right, left and biventricular forms of the disease, with underlying desmosomal and non-desmosomal mutations. Lifestyle physical activity, objectively monitored with accelerometers (ie, movement sensors) and RR-NSVT, identified as >188 bpm and >18 beats from a textile Holter ECG for 30 days.

Combination of late gadolinium enhancement and genotype improves prediction of prognosis in non-ischaemic dilated cardiomyopathy.

Aims: Genotype and left ventricular scar on cardiac magnetic resonance (CMR) are increasingly recognized as risk markers for adverse outcomes in non-ischaemic dilated cardiomyopathy (DCM). We investigated the combined influence of genotype and late gadolinium enhancement (LGE) in assessing prognosis in a large cohort of patients with DCM.

Methods And Results: Outcomes of 600 patients with DCM (53.

Heterozygous Arrhythmogenic Cardiomyopathy- Mutation Carriers Exhibit a Subclinical Cutaneous Phenotype with Cell Membrane Disruption and Lack of Intercellular Adhesion.

Genetic variants that result in truncation in () are a known cause of arrhythmogenic cardiomyopathy (AC). In homozygous carriers, the combined involvement of skin and heart muscle is well defined, however, this is not the case in heterozygous carriers. The aim of this work is to describe cutaneous findings and analyze the molecular and ultrastructural cutaneous changes in this group of patients.

Regression of Left Ventricular Hypertrophy in a Case of Sarcomeric Hypertrophic Cardiomyopathy: An Unexpected Outcome.

We present a case of sarcomeric hypertrophy cardiomyopathy diagnosed in a child who had hypertrophy degree regression during adolescence, with no left ventricular dysfunction and no increase of the ventricular diameters. ().