Short- and long-term outcomes in children undergoing radiofrequency catheter ablation before their second birthday

Background

Tachyarrhythmias can cause substantial morbidity and mortality in infants and very young children. Our objective was to assess early and late outcomes in children undergoing radiofrequency catheter ablation before their second birthday.

Methods

This is a retrospective review of medical records from 1995 till 2009 at a single institution with a large referral base.

Results

Thirty-four children younger than 2 years of age were brought to the electrophysiology laboratory for catheter ablation for tachyarrhythmia. Radiofrequency ablation (42 procedures) was performed in 31 children (mean weight, 7.4 kg; range, 2.6-12.3 kg). Tachyarrhythmias were atrioventricular re-entry tachycardia (19), ectopic or multifocal atrial tachycardia (6), atrial flutter (1), ventricular tachycardia (3), and congenital junctional ectopic tachycardia (2). Seventeen children presented with tachycardia-induced cardiomyopathy and heart failure. Three patients required extracorporeal life support prior to the procedure. Radiofrequency catheter ablation was successful in 74% of these very young children vs 91% in a comparison group of children older than 2 years (n = 447). Among patients with initially successful ablations, there was no tachyarrhythmia recurrence during medium- to long-term follow-up (1.4-15.0 years; mean, 7.3 years). Major complications occurred in 4 very young children. Eight of 11 patients in whom ablation failed or could not be done required another form of continued aggressive treatment.

Conclusion

Catheter ablation is indicated and successful in critically ill infants with life-threatening tachyarrhythmia refractory to medical therapy. Initially successful procedures are associated with excellent long-term outcomes, but significant complication rates require that such procedures be reserved for carefully selected patients and performed by highly skilled staff.     

Sudden Unexpected Death in Children with Heart Disease

Objective. To review a mortality database, and identify all sudden unexpected deaths in patients followed by the cardiac program. Design. Retrospective review of prospectively maintained database. Results. Over 8 years, we identified 80 sudden unexpected deaths, among which there were sufficient data in 69 (24 females). Patients died at a median age of 17.2 months (28 days?18.8 years). Forty‐six patients had 2 functional ventricles and 23 had received palliation for a single‐functional ventricle. Patients with a single ventricle died at a younger age (median 120 days; 28 days?17.2 years) and sooner after last assessment (median 27 days; 1–146 days) than patients in the biventricular group (median age 2 years; 43 days?18.8 years; median time since last assessment 49 days, 1 days?1 year) (P < .01; P = .01). Thrombosis was the most common cause (61%) of death in the single‐ventricle group. Arrhythmia or presumed arrhythmia was the most common cause (46%) of death in the biventricular group. Fifty‐one patients had undergone surgery. Six patients had primary electrophysiological disease, and 5 had cardiomyopathy. Eight deaths occurred in patients with pulmonary vascular disease. Conclusion. Our study demonstrates that sudden unexpected death occurred at a frequency of at least 10 patients per year over an 8‐year period with 55 730 patient encounters. We were able to determine a clinical cause of death in most patients. Arrhythmias (30%) and pulmonary vascular disease (13%) are important causes of sudden death. Simple aortic valve disease and hypertrophic cardiomyopathy are rare (4%) causes of sudden death in childhood. Infants and young children with surgical shunts comprise 23% of sudden unexpected deaths that occur within a month of the last evaluation. Close surveillance of these patients is warranted.     

Canadian Cardiovascular Society/Canadian Anesthesiologists' Society/Canadian Heart Rhythm Society joint position statement on the perioperative management of patients with implanted pacemakers, defibrillators, and neurostimulating devices

There are more than 200,000 Canadians living with permanent pacemakers or implantable defibrillators, many of whom will require surgery or invasive procedures each year. They face potential hazards when undergoing surgery; however, with appropriate planning and education of operating room personnel, adverse device-related outcomes should be rare. This joint position statement from the Canadian Cardiovascular Society (CCS) and the Canadian Anesthesiologists' Society (CAS) has been developed as an accessible reference for physicians and surgeons, providing an overview of the key issues for the preoperative, intraoperative, and postoperative care of these patients. The document summarizes the limited published literature in this field, but for most issues, relies heavily on the experience of the cardiologists and anesthesiologists who contributed to this work. This position statement outlines how to obtain information about an individual's type of pacemaker or implantable defibrillator and its programming. It also stresses the importance of determining if a patient is highly pacemaker-dependent and proposes a simple approach for nonelective evaluation of dependency. Although the document provides a comprehensive list of the intraoperative issues facing these patients, there is a focus on electromagnetic interference resulting from electrocautery and practical guidance is given regarding the characteristics of surgery, electrocautery, pacemakers, and defibrillators which are most likely to lead to interference. The document stresses the importance of preoperative consultation and planning to minimize complications. It reviews the relative merits of intraoperative magnet use vs reprogramming of devices and gives examples of situations where one or the other approach is preferable.     

The Medical Management of Pediatric Arrhythmias

OPINION STATEMENT: Arrhythmias are an important cause of morbidity and mortality in children. Despite recent technological advances in treatment, pharmacologic therapy remains the most common treatment modality for pediatric arrhythmias. The choice of antiarrhythmic agent, the duration of therapy, and the dosing schedule depend on multiple factors including the recurrence risk and the arrhythmia burden (the latter being determined by the hemodynamic effect of the arrhythmia), and the frequency and duration of episodes. As with all pediatric medications, consideration must be given to the drug formulation, palatability, adverse effects and adherence issues. There are very few randomized trials available to guide the choice of therapy for pediatric arrhythmias, and thus treatment options often reflect physician or institutional preferences. Although various classification schemes exist, we classify antiarrhythmic agents based on their primary site of action: atrial muscle/accessory pathway (class IA, IC, and III agents); the atrioventricular node (beta-blockers, calcium channel blockers, digoxin, and class III agents); or ventricular muscle (class I and III agents). This type of categorization assists in the approach to treatment required for each type of arrhythmia encountered.     

When the Blood Pressure Misleads You: A Diagnostic Conundrum in an Unusual Case of Coarctation

A 4-month-old previously healthy baby was found to be in congestive heart failure with LV dysfunction and a right aortic arch with severe coarctation, undetectable by blood pressure measurements. A cardiac CT and central blood pressure led to the diagnosis of a unique anatomic variant of aortic coarctation. Once diagnosed the patient underwent surgery with an uncomplicated recovery.     

Correction to: Faintly tired: a systematic review of fatigue in patients with orthostatic syncope

Clinical Autonomic Research -     

Society position statement

Purpose

There are more than 200,000 Canadians living with permanent pacemakers or implantable defibrillators, many of whom will require surgery or invasive procedures each year. They face potential hazards when undergoing surgery; however, with appropriate planning and education of operating room personnel, adverse device-related outcomes should be rare. This joint position statement from the Canadian Cardiovascular Society (CCS) and the Canadian Anesthesiologists?? Society (CAS) has been developed as an accessible reference for physicians and surgeons, providing an overview of the key issues for the preoperative, intraoperative, and postoperative care of these patients.

Principal findings

The document summarizes the limited published literature in this field, but for most issues, relies heavily on the experience of the cardiologists and anesthesiologists who contributed to this work. This position statement outlines how to obtain information about an individual??s type of pacemaker or implantable defibrillator and its programming. It also stresses the importance of determining if a patient is highly pacemaker-dependent and proposes a simple approach for nonelective evaluation of dependency. Although the document provides a comprehensive list of the intraoperative issues facing these patients, there is a focus on electromagnetic interference resulting from electrocautery and practical guidance is given regarding the characteristics of surgery, electrocautery, pacemakers, and defibrillators which are most likely to lead to interference.

Conclusions

The document stresses the importance of preoperative consultation and planning to minimize complications. It reviews the relative merits of intraoperative magnet use vs reprogramming of devices and gives examples of situations where one or the other approach is preferable.     

Minimally invasive approach to the child with palpitations

Palpitations are a common reason for referral to a pediatric cardiologist. Although generally benign, palpitations are a significant cause for concern in the individual and their family. Similarly, palpitations may be the initial presentation of significant heart disease, resulting in heightened concern in the referring physician. Although emphasis is usually placed on excluding arrhythmia as the cause for palpitations, there are a variety of noncardiac causes for palpitations. The patient history and physical examination are the key components of the evaluation and guide subsequent investigations. In many cases, an immediate diagnosis cannot be made and additional testing may be required; this often includes further monitoring for episodes, cardiac imaging and ambulatory monitoring. Current technologies for ambulatory monitoring during symptoms include Holter monitoring and a variety of patient-activated event recorders, including implantable loop recorders. Each presents its own unique advantages and disadvantages to aid diagnosis in the management of a child with palpitations. The primary focus for the clinician is to determine whether the etiology is benign in nature or whether there is underlying heart disease that may carry a more serious prognosis.