Recurrent immunosuppressive-responsive myocarditis in a patient with desmoplakin cardiomyopathy: a case report.

Background: Desmoplakin (DSP) cardiomyopathy is a rare genetic condition characterized by repeated inflammatory myocardial injury and is associated with ventricular arrhythmia and sudden cardiac death. Diagnosis is challenging and requires a combination of genetic testing and advanced imaging techniques.

Case Summary: We present the case of a 38-year-old woman with recurrent episodes of subclinical myocarditis.

Longitudinal assessment of structural phenotype in Brugada syndrome using cardiac magnetic resonance imaging.

Background: Despite historically being considered a channelopathy, subtle structural changes have been reported in Brugada syndrome (BrS) on histopathology and cardiac magnetic resonance (CMR) imaging. It is not known if these structural changes progress over time.

Objective: The study sought to assess if structural changes in BrS evolve over time with serial CMR assessment and to investigate the utility of parametric mapping techniques to identify diffuse fibrosis in BrS.

The role of genetic testing in diagnosis and care of inherited cardiac conditions in a specialised multidisciplinary clinic.

Background: The diagnostic yield of genetic testing for inherited cardiac diseases is up to 40% and is primarily indicated for screening of at-risk relatives. Here, we evaluate the role of genomics in diagnosis and management among consecutive individuals attending a specialised clinic and identify those with the highest likelihood of having a monogenic disease.

Methods: A retrospective audit of 1697 consecutive, unrelated probands referred to a specialised, multidisciplinary clinic between 2002 and 2020 was performed.

Cardiac Society of Australia and New Zealand (CSANZ) Position Statement on the Follow-Up of Cardiovascular Implantable Electronic Devices 2022.

Recognising the need for a national approach for the recommended best practice for the follow-up of implanted cardiac rhythm devices to ensure patient safety, this document has been produced by the Cardiac Society of Australia and New Zealand (CSANZ). It draws on accepted practice standards and guidelines of international electrophysiology bodies. It lays out methodology, frequency, and content of follow-up, including remote monitoring; personnel, including physician, allied health, nursing and industry; paediatric and adult congenital heart patients; and special considerations including magnetic resonance imaging scanning, perioperative management, and hazard alerts.

Atrial fibrillation management during septal myectomy for hypertrophic cardiomyopathy: A systematic review.

Introduction: Atrial fibrillation is common in patients with hypertrophic cardiomyopathy, and significantly impacts mortality and morbidity. In patients with atrial fibrillation undergoing septal myectomy, concomitant surgery for atrial fibrillation may improve outcomes.

Methods: A systematic review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

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.

Epidemiology and clinical characteristics of atrial fibrillation in patients with inherited heart diseases.

Background: The prevalence and clinical course of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) is well described, though less so for other inherited cardiomyopathies (familial dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular noncompaction); and inherited arrhythmia syndromes (long QT syndrome, Brugada syndrome or catecholaminergic polymorphic ventricular tachycardia [CPVT]). We examined the frequency, clinical characteristics and AF-related management and outcomes amongst this patient population.

Methods: We retrospectively studied consecutive probands with inherited cardiomyopathy (n = 962) and inherited arrhythmia syndromes (n = 195) evaluated between 2002 and 2018.

Twelve-lead ambulatory electrocardiographic monitoring in Brugada syndrome: Potential diagnostic and prognostic implications.

Background: Patients with Brugada syndrome (BrS) are diagnosed and risk stratified on the basis of a spontaneous or drug-induced type 1 electrocardiographic (ECG) pattern, often at single time points not accounting for variation throughout the day.

Objectives: The purpose of this study was to prospectively assess the overall burden of type 1 Brugada ECG changes using 12-lead 24-hour Holter monitoring and evaluate association with cardiac events.

Methods: From July 1, 2013 to December 31, 2015, patients with BrS were recruited from 3 Australian centers and the Australian Genetic Heart Disease Registry.

Cardiovascular Effects of Energy Drinks in Familial Long QT Syndrome: A Randomized Cross-Over Study.

Background: Caffeinated energy drinks may trigger serious cardiac effects. The aim of this study was to determine the cardiovascular effects of caffeinated energy drink consumption in patients with familial long QT syndrome (LQTS).

Methods And Results: From 2014-2016, 24 LQTS patients aged 16-50 years were recruited to a randomized, double-blind, cross-over study of energy drink (ED) versus control (CD) with participants acting as their own controls (one week washout).

Thoracoscopic surgical ablation versus catheter ablation for atrial fibrillation.

For patients with atrial fibrillation (AF) who are refractory to anti-arrhythmic drugs (AADs), minimally invasive video-assisted thoracoscopic surgical ablation (SA) and catheter ablation (CA) are potential alternative treatment options. The recent FAST randomized study suggested that thoracoscopic SA was superior to CA in achieving freedom of AF in patients who have failed at least one prior AAD. To assess the relative merits and risks of SA versus CA, a systematic review and meta-analysis was conducted.

Surgery for hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetically determined cardiac disease characterised by otherwise unexplained myocardial hypertrophy of the left ventricle, and may result in left ventricular outflow tract obstruction. It is the most common cause of sudden cardiac death in young adults due to arrhythmias. Septal myectomy is a surgical treatment for HCM with moderate to severe outflow tract obstruction, and is indicated for patients with severe symptoms refractory to medical therapy.

New-onset atrial fibrillation following coronary bypass surgery predicts long-term mortality: a systematic review and meta-analysis.

Atrial fibrillation (AF) is one of the most common postoperative complications following cardiac surgery. Recent evidence suggests that postoperative atrial fibrillation (POAF) may be more 'malignant' than previously thought, associated with follow-up mortality and morbidity. To evaluate the long-term survival of POAF versus No-POAF cohorts following coronary bypass surgery, the current meta-analysis with reconstructed individual patient data was performed.

Comparing energy sources for surgical ablation of atrial fibrillation: a Bayesian network meta-analysis of randomized, controlled trials.

Simplified maze procedures involving radiofrequency, cryoenergy and microwave energy sources have been increasingly utilized for surgical treatment of atrial fibrillation as an alternative to the traditional cut-and-sew approach. In the absence of direct comparisons, a Bayesian network meta-analysis is another alternative to assess the relative effect of different treatments, using indirect evidence. A Bayesian meta-analysis of indirect evidence was performed using 16 published randomized trials identified from 6 databases.

Prolongation of the QTc interval predicts appropriate implantable cardioverter-defibrillator therapies in hypertrophic cardiomyopathy.

Objectives: This study sought to determine factors predicting appropriate implantable cardioverter defibrillator (ICD) therapy in a large cohort of patients with hypertrophic cardiomyopathy (HCM).

Background: HCM is the leading cause of sudden cardiac death in those age ≤35 years. ICD therapy is offered to select patients at increased risk for sudden cardiac death.

Diffuse ventricular fibrosis is a late outcome of tachycardia-mediated cardiomyopathy after successful ablation.

Background: Successful arrhythmia ablation normalizes ejection fraction (EF) in tachycardia-mediated cardiomyopathy, but recurrent heart failure and late sudden death have been reported. The aim of this study was to characterize the left ventricle (LV) of tachycardia-mediated cardiomyopathy patients long after definitive arrhythmia cure.

Methods And Results: Thirty-three patients with a history of successfully ablated incessant focal atrial tachycardia 64±36 months prior, and 20 healthy controls were recruited.

Sinus rhythm restores ventricular function in patients with cardiomyopathy and no late gadolinium enhancement on cardiac magnetic resonance imaging who undergo catheter ablation for atrial fibrillation.

Background: Atrial fibrillation (AF) and systolic heart failure (HF) frequently coexist. Restoration of sinus rhythm by catheter ablation may result in a variable improvement in left ventricular (LV) function. Late-gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging identifies irreversible structural change and may predict incomplete recovery of LV function.

Subtle post-procedural cognitive dysfunction after atrial fibrillation ablation.

Objectives: This study sought to determine whether post-operative neurocognitive dysfunction (POCD) occurs after ablation for atrial fibrillation (AF).

Background: Ablation for AF is a highly effective strategy; however, the risk of transient ischemic attack and stroke is approximately 0.5% to 1%.

Left septal atrial tachycardias: electrocardiographic and electrophysiologic characterization of a paraseptal focus.

Objective: The objective was to characterize the electrocardiographic and electrophysiological features of focal atrial tachycardia (FAT) originating from the left septum (LS).

Background: FAT is recognized to occur at predefined anatomic locations rather than randomly throughout the atria. We describe the ECG and EP features of ATs originating from the LS as an important site for apparent perinodal tachycardias.

Atrial remodeling in varying clinical substrates within beating human hearts: relevance to atrial fibrillation.

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia in human beating hearts. AF initiates self-perpetuating changes in electrophysiology, structure and functional properties of the atria, a phenomenon known as atrial remodeling. Hypertension, heart failure, valvular heart disease, sleep apnea, congenital heart disease are well known risk factors for AF that contribute to the development of atrial substrate.

Right atrial remodeling is more advanced in patients with atrial flutter than with atrial fibrillation.

Introduction: Atrial fibrillation (AF) and atrial flutter (AFL) are related arrhythmias with common triggers, yet in individual patients either AF or AFL often predominates. We performed detailed electrophysiologic (EP) and electroanatomic (EA) studies of the right atrium (RA) in patients with AF and AFL to determine substrate differences that may explain the preferential expression of AF/AFL in individual patients.

Methods: Patients with AF (n = 13) were compared to patients with persistent AFL (n = 10).

Atrial electrical and structural remodeling associated with longstanding pulmonary hypertension and right ventricular hypertrophy in humans.

Introduction: Pulmonary hypertension (PH) is common to a range of cardiopulmonary conditions and is associated with atrial arrhythmias. However, little is known of the isolated atrial effects of PH and right atrial dilatation (RA) in humans. To avoid the confounding effects of PH-associated disease states, we performed detailed electrophysiological (EP) and electroanatomic (EA) mapping of the RA in patients with idiopathic PH.

Long-term effects of catheter ablation for lone atrial fibrillation: progressive atrial electroanatomic substrate remodeling despite successful ablation.

Background: Whether curative ablation can prevent progression of the atrial electroanatomic remodeling associated with atrial fibrillation (AF) is not known.

Objective: The purpose of this study was to determine whether successful radiofrequency ablation (RFA) of AF can prevent progression of the atrial substrate associated with AF.

Methods: Detailed right atrial electroanatomic maps from 11 patients without apparent structural heart disease undergoing RFA of AF at baseline and ≥6 months following successful RFA were compared to 11 control patients undergoing electrophysiologic evaluation of supraventricular tachycardia.