Evolution of Right Ventricular Electromechanical Dyssynchrony during Childhood after Tetralogy of Fallot Repair.

Background: Patients with repaired tetralogy of Fallot (rTOF) develop electromechanical dyssynchrony (EMD), which detrimentally affects right ventricular (RV) function and exercise capacity. However, EMD evolution over childhood is unknown.

Methods: We retrospectively studied serial ECG, Holter, echocardiography, cardiac magnetic resonance, and exercise data of rTOF patients, over the first 18-years of life, who underwent repair between 2010-2020.

Epicardial Devices in Pediatrics and Congenital Heart Disease.

Epicardial cardiac implantable electronic device implant remains a common option in pediatric patients and certain patients with congenital heart disease due to patient size, complex anatomy, residual intracardiac shunts, and prior surgery precluding transvenous implant. Advantages include the lack of thromboembolic and vascular risks and ability to implant during concomitant surgery. Significant disadvantages include the occurrence of lead dysfunction that can result in bradycardia events in pacemaker patients, inappropriate shocks in implantable cardiac defibrillator patients, and overall a more invasive procedure.

Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation.

Background: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPM) may be associated with additional adverse long-term outcomes.

Objectives: The goal of this study was to quantify the attributable risk of PPM in patients with SV, and to identify modifiable risk factors.

Successful treatment of severe hypertriglyceridemia with icosapent ethyl in a case of congenital generalized lipodystrophy type 4.

Objectives: Congenital generalized lipodystrophy type 4 (CGL4) is a rare autosomal recessive condition with high rates of morbidity and mortality. It is a multisystem condition associated with ventricular tachyarrhythmia, congenital myopathy, hepatitis, and metabolic profile of severe hypertriglyceridemia and insulin resistance. Metreleptin is the first line treatment, however it is unavailable in several countries.

Relation of Norwood Shunt Type and Frequency of Arrhythmias at 6 Years (from the Single Ventricle Reconstruction Trial).

The Norwood procedure with a right ventricular to pulmonary artery shunt (RVPAS) decreases early mortality, but requires a ventriculotomy, possibly increasing risk of ventricular arrhythmias (VAs) compared with the modified Blalock-Taussig shunt (MBTS). The effect of shunt and Fontan type on arrhythmias by 6 years of age in the SVRII (Single Ventricle Reconstruction Extension Study) was assessed. SVRII data collected on 324 patients pre-/post-Fontan and annually at 2 to 6 years included antiarrhythmic medications, electrocardiography (ECG) at Fontan, and Holter/ECG at 6 years.

Ventricular pacing in single ventricles-A bad combination.

Background: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population.

Objective: To determine the longitudinal effect of VP in SV patients.

B cell receptor-induced phosphorylation of Pyk2 and focal adhesion kinase involves integrins and the Rap GTPases and is required for B cell spreading.

Signaling by the B cell receptor (BCR) promotes integrin-mediated adhesion and cytoskeletal reorganization. This results in B cell spreading, which enhances the ability of B cells to bind antigens and become activated. Proline-rich tyrosine kinase (Pyk2) and focal adhesion kinase (FAK) are related cytoplasmic tyrosine kinases that regulate cell adhesion, cell morphology, and cell migration.