Updates on inherited arrhythmia syndromes (Brugada syndrome, long QT syndrome, CPVT, ARVC).

The inherited arrhythmia (IA) syndromes are a group of rare and complex conditions that may predispose individuals to ventricular arrhythmias and sudden cardiac death. Our understanding of the genetic architecture underlying these syndromes has evolved, with recent reappraisals of variant pathogenicity and quantification of polygenic influences. The IA population includes an increasing proportion of low-risk patients, often identified via familial screening; avoiding over-treatment in these patients is an important consideration.

Outcomes of permanent pacemakers and implantable cardioverter-defibrillators in an adult congenital heart disease population.

Background: Brady- and tachyarrhythmias commonly complicate adult congenital heart disease (ACHD). Permanent pacemakers (PPMs) or implantable cardioverter-defibrillators (ICDs) are often utilised to prevent morbidity or mortality related to arrhythmia, but can also be associated with significant morbidity themselves.

Methods: We analysed outcomes from patients in our comprehensive ACHD database who were seen at least twice since 2000 and once since 2018.

Noninvasive ECG imaging of the intrinsic atrial pacemaker and atrial activation in surgically repaired or palliated congenital heart disease.

Introduction: Sinus node location, function, and atrial activation are often abnormal in patients with congenital heart disease (CHD), due to anatomical, surgical, and acquired factors. We aimed to perform noninvasive electrocardiographic imaging (ECGI) of the intrinsic atrial pacemaker and atrial activation in patients with surgically repaired or palliated CHD, compared with control patients with structurally normal hearts.

Methods And Results: Atrial ECGI was performed in eight CHD patients with prespecified diagnoses (Fontan circulation, dextro transposition of the great arteries post Mustard/Senning, tetralogy of Fallot), and three controls.

His bundle combined with deep septal left bundle branch area pacing for atrial fibrillation prior to atrioventricular node ablation.

Background: To mitigate the risk of dyssynchrony-induced cardiomyopathy, international guidelines advocate His bundle pacing (HBP) with a ventricular backup lead prior to atrioventricular node ablation in treatment-refractory atrial fibrillation and normal left ventricular ejection fraction. As a result of concerns with long-term pacing parameters associated with HBP, this case series reports an adopted strategy of HBP combined with deep septal left bundle branch area pacing (dsLBBAP) in this patient cohort, enabling intrapatient comparison of the two pacing methods.

Methods And Results: Eight patients aged 72 ± 10 years (left ventricular ejection fraction 53 ± 4%) underwent successful combined HBP and dsLBBAP implant prior to AV node ablation.

Higher power short duration vs. lower power longer duration posterior wall ablation for atrial fibrillation and oesophageal injury outcomes: a prospective multi-centre randomized controlled study (Hi-Lo HEAT trial).

Aims: Radiofrequency (RF) ablation for pulmonary vein isolation (PVI) in atrial fibrillation (AF) is associated with the risk of oesophageal thermal injury (ETI). Higher power short duration (HPSD) ablation results in preferential local resistive heating over distal conductive heating. Although HPSD has become increasingly common, no randomized study has compared ETI risk with conventional lower power longer duration (LPLD) ablation.

Sudden cardiac death in congenital heart disease.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity.

Pacing-associated cardiomyopathy in adult congenital heart disease.

Objectives: Long-term single-site ventricular pacing may adversely affect ventricular function, due to dyssynchronous systemic ventricular contraction. We sought to determine the incidence, predictors and outcomes of pacing-associated cardiomyopathy (PACM) in an adult congenital heart disease (ACHD) cohort.

Methods: We retrospectively identified all patients in our database with a permanent pacemaker from 2000 to 2019.

Defibrillators in adult congenital heart disease: Long-term risk of appropriate shocks, inappropriate shocks, and complications.

Aims: Sudden cardiac death (SCD) accounts for up to 25% of deaths in the adult congenital heart disease (ACHD) population. Current guidelines for defibrillator implantation are either extrapolated from acquired cardiac disease or are based upon single lesion studies, predominantly Tetralogy of Fallot (TOF). Defibrillator-related morbidity appears to be substantially higher in ACHD patients.

Rugby Player's Aorta: Alarming Prevalence of Ascending Aortic Dilatation and Effacement in Elite Rugby Players.

Background: Prompted by a cluster of observations concerning ascending aortic pathology in elite rugby players, we assessed over 150 asymptomatic predominantly retired players with echocardiography, aiming to document the prevalence and severity of ascending aortic dilatation and/or anterior aortic effacement, both 'risk factors' for potentially catastrophic aortic complications.

Methods: Rugby players (at least 5 years of high level competitive rugby) were classified as elite (national, state or first grade representatives) or non-elite. A total of 152 asymptomatic players with a mean age of 45 ± 13 years (range 21-65) underwent transthoracic echocardiography.

Ablation of Atrial Arrhythmias After the Atriopulmonary Fontan Procedure: Mechanisms of Arrhythmia and Outcomes.

Objectives: This study sought to describe atrial arrhythmia mechanisms, acute outcomes, and long-term arrhythmia burdens following catheter ablation in adult atriopulmonary (AP) Fontan patients.

Background: Atrial arrhythmias are a significant cause of morbidity and mortality in the AP Fontan population.

Methods: Sixty consecutive atrial arrhythmia ablations were reviewed in 42 AP Fontan patients (31 ± 8 years of age), performed between 1998 and 2017.

Adverse effects of amiodarone therapy in adults with congenital heart disease.

Objective: Amiodarone is a highly effective antiarrhythmic therapy, however its toxicity profile often limits treatment. This is particularly relevant in adults with congenital heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD).

Efficacy and adverse effects of sotalol in adults with congenital heart disease.

Introduction: Adults with congenital heart disease (CHD) are predisposed to arrhythmias, which can often be refractory to medical therapy. Sotalol is an attractive alternative antiarrhythmic to amiodarone in this younger patient population, given the latter's toxicity profile, but it may have proarrhythmic effects. We therefore aimed to assess the efficacy and safety of sotalol in adults with CHD.

Causes of death in a contemporary adult congenital heart disease cohort.

Objective: The life expectancy of patients with congenital heart disease (CHD) has significantly improved with advances in their paediatric medical care. Mortality patterns are changing as a result. Our study aims to describe survival and causes of death in a contemporary cohort of adult patients with CHD.

Impact of Perturbed Pancreatic β-Cell Cholesterol Homeostasis on Adipose Tissue and Skeletal Muscle Metabolism.

Elevated pancreatic β-cell cholesterol levels impair insulin secretion and reduce plasma insulin levels. This study establishes that low plasma insulin levels have a detrimental effect on two major insulin target tissues: adipose tissue and skeletal muscle. Mice with increased β-cell cholesterol levels were generated by conditional deletion of the ATP-binding cassette transporters, ABCA1 and ABCG1, in β-cells (β-DKO mice).

The impact of nerve sparing on incidence and location of positive surgical margins in radical prostatectomy.

Objective: •  To examine whether nerve-sparing surgery (NSS) is a risk factor for positive surgical margins (PSMs) in patients with either organ-confined prostate cancer or extracapsular extension (ECE).

Patients And Methods: •  Clinicopathological outcome data on 945 consecutive patients treated with radical prostatectomy (RP) were prospectively collected. •  All patients underwent RP (bilateral, unilateral or non-NSS) by one surgeon between 2002 and 2007.