Four cardiomyopathy patients with a heterozygous DSG2 p.Arg119Ter variant.

DSG2, encoding desmoglein-2, is one of the causative genes of arrhythmogenic cardiomyopathy. We previously identified a homozygous DSG2 p.Arg119Ter stop-gain variant in a patient with juvenile-onset cardiomyopathy and advanced biventricular heart failure.

A case report of pre-implantation feasibility test for combining leadless pacemaker and subcutaneous implantable cardioverter-defibrillator in adult congenital heart disease.

Unlabelled: New cardiac implantable electronic devices (CIEDs), such as leadless pacemakers and subcutaneous implantable cardioverter defibrillators (S-ICDs), are being used in patients with adult congenital heart disease. The selection of CIEDs often requires careful consideration due to technical challenges posed by a unique heart structure. A 27-year-old man following a surgical tetralogy of Fallot (TOF) repair developed non-sustained ventricular tachycardia, sick sinus syndrome, and complete atrioventricular block.

Unusual resetting of superior-type fast-slow atrioventricular nodal reentrant tachycardia with cycle length alternans by early spontaneous premature ventricular contraction.

The pacing maneuvers for supraventricular tachycardia with cycle length alternans are sometimes difficult, especially when diagnostic ventricular pacing does not conduct to the atrium. Even in such a situation, critical diagnostic findings can be obtained by spontaneous premature ventricular contraction.

Paradoxical generator impedance behavior during catheter ablation in a patient with severe polycythemia.

An extremely high generator impedance in the blood pool can be observed in a patient with severe polycythemia. However, ablation can be performed safely as long as the generator impedance during contact with the myocardial tissue is within acceptable limits.

Recurrent Pericardial Effusion Resulting From Right Ventricular Free Wall Injury Caused by Leadless Pacemaker Tines.

An 87-year-old man developed delayed cardiac tamponade 55 min after leadless pacemaker implantation and recurrent pericardial effusion 20 days later. Electrocardiogram-gated enhanced cardiac computed tomography revealed that the leadless pacemaker tines on the lateral side had penetrated the right ventricular free wall. He underwent off-pump hemostatic surgery.

Appropriate Selection of Substrate Ablation for Persistent Atrial Fibrillation Using Intraprocedural Assessment.

Background: It has not been fully elucidated which patients with persistent atrial fibrillation (PerAF) should undergo substrate ablation plus pulmonary vein isolation (PVI). This study aimed to identify PerAF patients who required substrate ablation using intraprocedural assessment of the baseline rhythm and the origin of atrial fibrillation (AF) triggers.

Methods and results: This was a post hoc subanalysis using extended data of the EARNEST-PVI trial, a prospective multicenter randomized trial comparing PVI-alone and PVI-plus (i.

Impact of left atrial appendage flow velocity on thrombus resolution and clinical outcomes in patients with atrial fibrillation and silent left atrial thrombi: insights from the LAT study.

Aims: Blood stasis is crucial in developing left atrial (LA) thrombi. LA appendage peak flow velocity (LAAFV) is a quantitative parameter for estimating thromboembolic risk. However, its impact on LA thrombus resolution and clinical outcomes remains unclear.

Quantitative analysis of fractionated electrogram area of left atrium during right atrial pacing as an indicator of left atrial electrical remodeling in patients with atrial fibrillation.

Background: The clinical significance of left atrial local electrogram fractionation after restoration of sinus rhythm in patients with atrial fibrillation (AF) has not been elucidated.

Methods: We evaluated ultrahigh-resolution maps of the left atrium (LA) during RA pacing acquired after pulmonary vein isolation in 40 patients with AF. The association between low-voltage area (LVA, <0.

Complete free wall isolation of arrhythmogenic persistent left superior vena cava.

Introduction: Persistent left superior vena cava (PLSVC) is one of the major sources of triggers and drivers of atrial fibrillation (AF). There has been no established PLSVC ablation procedure to eliminate the arrhythmogenicity along the entire length of PLSVC.

Methods And Results: A 70-year-old woman with a history of two previous catheter ablations for AF, mitral valvuloplasty, and an unroofed coronary sinus-type atrial septal defect closure underwent the redo AF ablations.

Recurrence as isthmus-related atrial tachycardia: A cautionary point of low-voltage area homogenization of persistent atrial fibrillation.

Unlabelled: A 66-year-old female underwent persistent atrial fibrillation ablation. After pulmonary vein isolation and homogenization of low-voltage areas (LVAs), atrial tachycardia (AT) was not induced at the first session; however, it recurred one year after the procedure. During the second session, the extensive LVAs were distributed in the same area of the left atrial anterior wall and expanded possibly due to the previous LVA homogenization.

Long-Term Impact of Additional Ablation After Pulmonary Vein Isolation: Results From EARNEST-PVI Trial.

Background An optimal strategy for left atrial ablation in addition to pulmonary vein isolation (PVI) in patients with persistent atrial fibrillation (AF) has not been determined. Methods and Results We conducted an extended follow-up of the multicenter randomized controlled EARNEST-PVI (Efficacy of Pulmonary Vein Isolation Alone in Patients With Persistent Atrial Fibrillation) trial, which compared 12-month rhythm outcomes in patients with persistent AF between patients randomized to a PVI-alone strategy (n=248) or PVI-plus strategy (n=248; PVI followed by left atrial additional ablation, including linear ablation or ablation targeting areas with complex fractionated electrograms). The present study extended the follow-up period to 3 years after enrollment.

Lysophosphatidylserine induces necrosis in pressure overloaded male mouse hearts via G protein coupled receptor 34.

Heart failure is a leading cause of mortality in developed countries. Cell death is a key player in the development of heart failure. Calcium-independent phospholipase Aβ (iPLAβ) produces lipid mediators by catalyzing lipids and induces nuclear shrinkage in caspase-independent cell death.

Left atrial anterolateral linear ablation for biatrial tachycardia via Bachmann's bundle, interatrial septum, and left atrial anterior wall under mitral isthmus block.

Biatrial tachycardia via Bachmann's bundle, interatrial septum, and left atrial anterior wall can be treated by left atrial anterolateral linear ablation without left atrial appendage isolation, even under mitral isthmus block.

Delineation of an intra-isthmus reentry circuit around the coronary sinus ostium using an ultrahigh-resolution mapping system.

• Intra-isthmus reentry is a variant of cavotricuspid isthmus-dependent atrial flutter. • Intra-isthmus reentry circuit can involve the rotational activation around the coronary sinus ostium. • Ultrahigh-resolution mapping can help delineate the complete intra-isthmus reentry circuit.