Cardiac Electrophysiology Clinics

Subcutaneous implantable cardioverter defibrillators (S-ICDs) are being used with increased frequency in children and patients with congenital heart disease. Vascular access complexities, intracardiac shunts, and specific anatomies make these devices particularly appealing for some of these patients. Alternative screening, implantation, and programming techniques should be considered based on patient size, body habitus, anatomy, procedural history, and preference. Appropriate and inappropriate shock rates are generally comparable to those seen with transvenous devices...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Pediatric electrophysiologists believe that there is a paucity of pediatric-specific cardiac implantable electronic devices (CIEDs) available for their patients. Specific patient characteristics such as vascular size, intracardiac anatomy, and expected somatic growth limit the types of CIED implants possible for pediatric and congenital heart disease (CHD) patients. These patients demonstrate higher CIED-related complication rates compared with adults. As the number of pediatric and CHD patients who require CIEDs increases, so does the need for advocacy...

This article reviews various opportunities to translate established and novel tools and techniques used in adult electrophysiology to pediatrics and the adult congenital heart disease population. There is a specific focus on preoperative management of special population, implantation techniques, and postoperative programming of devices.

Pediatric and congenital heart disease patients may require cardiac implantable electronic device implantation, inclusive of pacemaker, ICD, and implantable cardiac monitor, for a variety of etiologies. While leads, generators, and monitors have decreased in size over the years, they remain less ideal for the smallest patients. The potential for a miniature pacemaker, fetal micropacemaker, improving leadless technology, and rechargeable devices creates hope that the development of pediatric-focused devices will increase...

Risk stratification for sudden death should be discussed with patients with congenital heart disease at each stage of personal and cardiac development. For most patients, risk is low through teenage years and the critical factors to consider are anatomy, ventricular function, and symptoms. By adulthood, these are supplemented by screening for atrial arrhythmias, ventricular arrhythmias, and pulmonary hypertension. Therapies include medication, ablation, and defibrillator placement.

Pediatric patients with congenital heart disease present unique challenges when it comes to cardiac implantable electronic devices. Pacing strategy is often determined by patient size/weight and operator experience. Anatomic considerations, including residual shunts, anatomic obstructions and barriers, and abnormalities in the native conduction system, will affect the type of CIED implanted. Given the young age of patients, it is important to have an "eye on the future" when making pacemaker/defibrillator decisions, as one can expect several generator changes, lead revisions, and potential lead extractions during their lifetime...

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...

For patients with congenital heart disease (CHD), chronic ventricular pacing may lead to progressive cardiomyopathy owing to electromechanical dyssynchrony. Cardiac conduction system pacing (CSP) has been proposed as a physiologic pacing strategy-directly engaging the His-Purkinje system and preserving electromechanical synchrony. CSP may be indicated for a wide variety of children and adults with CHD and has emerged as an important tool in the armamentarium for cardiac implantable electronic device operators...

Cardiac resynchronization therapy (CRT) for congenital heart disease has shown promising suucess as an adjunct to medical therapy for heart failure. While cardiac conduction defects and need for ventricular pacing are common in congential heart disease, CRT indications, techniques and long term outcomes have not been well establaished. This is a review of the techniques nad short term outcomes of CRT for the following complex congenital heart disease conditions: single ventricle physiology, systemic right ventricle, and the subpulmonic right ventricle...

Heart failure in patients with congenital heart disease (CHD) stems from unique causes compared with the elderly. Patients with CHD face structural abnormalities and malformations present from birth, leading to altered cardiac function and potential complications. In contrast, elderly individuals primarily experience heart failure due to age-related changes and underlying cardiovascular conditions. Cardiac resynchronization therapy (CRT) can benefit patients with CHD, although it presents numerous challenges...

Transcatheter leadless pacemakers have benefits in congenital heart disease because they eliminate the risks of lead malfunction, venous occlusions, and pocket complications. This newest pacemaker's utility in this population has been limited by the large sheath and delivery system, need for atrioventricular synchronous pacing, lack of explantation options, and possible lack of adequate access to the subpulmonary ventricle. With careful planning, leadless pacing can be successfully performed in these patients...

Insertable cardiac monitors (ICMs) have been used more frequently and in a wider variety of circumstances in recent years. ICMs are used for symptom-rhythm correlation when patients have potentially arrhythmogenic syncope and for less traditional reasons such as rhythm surveillance in patients with genetic arrhythmia syndromes or other diseases with high arrhythmia risk. ICMs have good diagnostic yield in pediatric patients and in adults with congenital heart disease and have a low rate of complications. Implantation techniques should take patient-specific factors into account to optimize diagnostic yield and minimize risk...

Surgery for congenital heart disease may compromise atrioventricular (AV) nodal conduction, potentially resulting in postoperative AV block. In the majority of cases, AV nodal function recovers during the early postoperative period and may only require short-term pacing support, typically provided via temporary epicardial wires. Permanent pacing is indicated when the postoperative AV block persists for more than 7 to 10 days due to the risk of mortality if a pacemaker is not implanted. Although there is a subset of patients who may have late recovery of AV nodal function, those with continued postoperative AV block will need lifelong pacing therapy...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Arrhythmogenic left ventricular cardiomyopathy is characterized by early malignant ventricular arrhythmia associated with varying degrees and times of onset of left ventricular dysfunction. Variants in numerous genes have been associated with this phenotype. Here, the authors review the literature on recent cohort studies of patients with variants in desmoplakin, lamin A/C, filamin-C, phospholamban, RBM20, TMEM43, and selected channelopathy genes also associated with structural disease. Unlike traditional sudden cardiac death risk assessment in nonischemic cardiomyopathy, left ventricular systolic function is an insensitive predictor of risk in patients with these genetic diagnoses...

Left ventricular ejection fraction-based arrhythmic risk stratification in nonischemic cardiomyopathy (NICM) is insufficient and has led to the failure of primary prevention implantable cardioverter defibrillator trials, mainly due to the inability of selecting patients at high risk for sudden cardiac death (SCD). Cardiac magnetic resonance offers unique opportunities for tissue characterization and has gained a central role in arrhythmic risk stratification in NICM. The presence of myocardial scar, denoted by late gadolinium enhancement, is a significant, independent, and strong predictor of ventricular arrhythmias and SCD with high negative predictive value...