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.     

Level and direction of hope in cancer patients: an exploratory longitudinal study

Goals

Hope is an important factor to consider when caring for cancer patients as a key component of coping with adversity. The aim of our study was to address the following questions: Is there a difference in level of hope between those patients being curatively and those palliatively treated, and how does this change over time? What are patients’ most important hopes? Better understanding of patients’ hopes may promote more effective patient-centered care.

Materials and methods

Outpatients referred to medical oncology or pain and symptom clinics at the London Regional Cancer Program were surveyed before consultation. The Herth Hope Index (HHI) questionnaire was administered to assess level of hope, and the patients were asked to indicate their highest priority hopes. Qualitative thematic analysis was performed on the responses, and comparison was made between patients treated with curative vs those treated with palliative intent. This survey was repeated 4 months after initial assessment.

Results

Fifty patients were surveyed (29 curative-intent; 21 palliative). Highest priority initial hopes were categorized as follows: cure, other positive health outcomes, emotional well-being, life achievements/return to normalcy, interpersonal goals, other. There was no association noted between treatment intent and choice of highest-priority hope. Follow-up assessments after 4 months revealed no significant differences in the distribution pattern of hopes. There was a statistically significant increase in the HHI over time in curatively treated patients, but none in the combined analysis.

Discussion and conclusion

Our study indicates that patients receiving palliative therapy have a HHI score not significantly different from patients being treated for cure. The hope deemed most important is also similar between groups. Over time, overall hope was maintained or increased even in the presence of a trend towards fewer patients hoping for a cure. These results remind oncologists to explore the experience of hope with all patients to ensure that the subjective needs and goals of the patients are met by the proposed therapies.

     

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

Clinical Autonomic Research -     

Near Miss or Standard of Care? DPYD Screening for Cancer Patients Receiving Fluorouracil

5-fluorouracil (5-FU) and its pro-drug capecitabine are widely used anticancer agents. Most 5-FU catabolism is dependent on dihydropyrimidine dehydrogenase (DPD) encoded by the DPYD gene, and DPYD variants that reduce DPD function increase 5-FU toxicity. Most DPD deficient patients are heterozygous and can be treated with reduced 5-FU dosing. We describe a patient with a genotype associated with near complete absence of DPD function, and severe and likely fatal toxicity with 5-FU treatment. The patient was treated effectively with alternative systemic therapy. Routine pretreatment DPYD genotyping is recommended by the European Medicines Agency, and guidelines for use of 5-FU in DPD deficient patients are available. However, outside the province of Quebec, routine pretreatment screening for DPD deficiency remains unavailable in Canada. It is likely our patient would have died from 5-FU toxicity under the current standard of care, but instead provides an example of the potential benefit of DPYD screening on patient outcomes.