Congenital coronary artery anomalies represent a rare cause for cardiac arrest in children and adults; however, most of these anomalies are asymptomatic and incidental findings. We report on a 14-year-old boy who was admitted to our hospital after cardiopulmonary resuscitation at home. Diagnostic workup including histopathology revealed parvovirus B19 in endomyocardial biopsy. Moreover, cardiac catheterisation as well as CT angiography identified an anomalous origin of the right coronary artery with an interarterial course. Since this anomalous coronary artery might have caused impaired myocardial perfusion causing cardiac arrest, surgical correction and implantation of a cardioverter defibrillator were performed. The further post-operative clinical course (7 months) has been uneventful.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff–Parkinson–White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Upon his refusal to receive implantable cardioverter/defibrillator therapy, we ablated anatomical arrhythmogenic substrate instead, and he remained uneventfully over 3 years on β-blocker.

As the population ages and cardiovascular disease becomes more prevalent, an increasing number of patients are receiving implantable cardioverter-defibrillators (ICDs). When these patients present to the emergency department, it is imperative that physicians are not only aware of the possible underlying medical issues that may have precipitated their admission but should also have a good understanding of the potential interactions that any medical intervention may have on the patient’s device. We discuss a case in which a patient known to have an ICD in situ was transcutaneously paced for the management of bradycardia, leading to an unnecessary shock.

Objectives: Implantable cardioverter defibrillator (ICD) therapy reduces the risk of sudden death in patients with ischemic cardiomyopathy, but their novelty and cost may represent barriers to utilization. The objective of this study was to examine the influence of age, gender, place of residence, and socioeconomic status on rates of ICD implantation for the primary prevention of death.

Methods: We conducted a population-based retrospective cohort study involving the entire province of Ontario, Canada. Patients were eligible if they had survived following hospitalization for heart failure from 1 January 1993, to 31 March 2004, and previously sustained an acute coronary syndrome within 5 years. Patients with an existing ICD or a documented history of cardiac arrest were excluded, as were patients who died in the hospital. Primary outcome was ICD implantation.

Results: We identified 48,426 patients hospitalized for heart failure who survived to hospital discharge. Of these, 440 received an ICD, with a gradual 30-fold increase in implantation rates over the study period (.12–3.9 percent). ICD recipients were more likely to be men (odds ratio [OR] = 4.14; 95 percent confidence interval [CI], 3.24–5.30), younger than 75 years of age (OR = 3.19; 95 percent CI, 2.57–3.96), reside in a metropolitan area (OR = 1.42; 95 percent CI, 1.04–1.9), and live in a higher socioeconomic neighborhood (OR = 1.32; 95 percent CI, 1.08–1.61).

Conclusions: Among patients with heart failure and a previous myocardial infarction, ICD use is increasing in Ontario. However, the application of this technology is characterized by major sociodemographic inequities. The causes and consequences of the pronounced age and gender discrepancies, in particular, warrant further investigation.

Objectives: The clinical effectiveness and cost-effectiveness of implantable cardioverter defibrillators (ICD) for arrhythmias was assessed.

Methods: A systematic review of the literature of systematic reviews and randomized controlled trials that reported mortality outcomes associated with implantable cardioverter defibrillators compared with antiarrhythmic drug therapy in people at risk of sudden cardiac death due to arrhythmias was undertaken. Economic evaluations were also sought. Inclusion criteria, data extraction, and quality assessment were undertaken by standard methodology. A decision analytic model was constructed using best available evidence to determine cost-effectiveness in a UK setting.

Results: Eight randomized controlled trials, two systematic reviews, and a meta-analysis met the inclusion criteria and were of variable quality. Evidence suggests that ICDs reduce mortality in both secondary and primary prevention, although the magnitude of benefit depends on baseline risk for sudden cardiac death. Incremental cost per quality-adjusted life year ranged from £52,000 ($98,000) to over £200,000 ($379,000), depending on mortality risk and assumptions made.

Conclusions: Evidence suggests that ICDs reduce total mortality but may be cost-effective only in some subgroups of patients at high risk of ventricular arrhythmias. Further research is needed on risk stratification of patients in whom ICDs are most likely to be clinically and cost-effective.