Anoop K. Singh

Impact of Cardiac Devices on the Quality of Life in Pediatric Patients

Background— Cardiac rhythm devices are increasingly used in the pediatric population, although their impact on quality of life (QOL) is poorly understood. The purpose of this study was to compare (QOL) scores among pediatric device patients, healthy controls, and congenital heart disease (CHD) patients and determine the key drivers of QOL in pediatric device patients. Methods and Results— Multicenter, cross-sectional study at 8 pediatric centers of subjects aged 8 to 18 years with either a pacemaker or defibrillator was carried out. Patient–parent pairs completed the Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory. QOL outcomes in device patients were compared with healthy controls and patients with various forms of CHD. Structural equation modeling was used to test for differences in Pediatric Cardiac Quality of Life Inventory scores among (1) device type, (2) presence of CHD, and (3) hypothesized key drivers of QOL. One hundred seventy-three patient–parent pairs (40 defibrillators/133 pacemakers) were included. Compared with healthy controls, patients with devices and their parents reported significantly lower Pediatric Quality of Life Inventory scoring. Similarly, compared with patients with mild forms of CHD, parents and patients with devices reported significantly lower Pediatric Cardiac Quality of Life Inventory scores and were similar to patients with more severe CHD. Key drivers of patient QOL were presence of implantable cardioverter-defibrillator and CHD. For patients, self-perception was a key driver of lower QOL, whereas for parents behavioral issues were associated with lower QOL. Conclusions— Patient QOL is significantly affected by the presence of cardiac rhythm devices. Whether these effects can be mitigated through the use of psychotherapy needs to be assessed.

Current Management of Focal Atrial Tachycardia in Children A Multicenter Experience

Background—Focal atrial tachycardia (FAT) is an uncommon cause of supraventricular tachycardia in children. Incessant FAT can lead to tachycardia-induced cardiomyopathy. There is limited information regarding the clinical course and management of FAT. This study characterizes current management strategies for FAT in children including the prevalence of spontaneous resolution and the role of catheter ablation. Methods and Results—This is a retrospective chart review of pediatric patients with FAT managed between January 2000 and November 2010 at 10 pediatric centers. There were 249 patients with a median age at diagnosis of 7.2 (95% confidence interval, 5.8–10.4) years. Cardiomyopathy was observed in 28%. Resolution of FAT occurred in 89%, including spontaneous resolution without catheter ablation in 34%. Antiarrhythmic medications were used for initial therapy in 154 patients with control of FAT in 72%. Among first-line medications, &bgr;-blockers were the most common (53%) and effective (42%). Catheter ablation was successful in 80% of patients. FAT recurrence was less common with electroanatomic mapping compared with conventional mapping techniques (16% versus 35%; P=0.02). Patients were followed for a median of 2.1 (95% confidence interval, 1.8–2.6) years. Conclusions—FAT is managed successfully in most children. Current approaches are variable. Many patients have control of FAT with medications; however, catheter ablation is used for most patients. Spontaneous resolution is common for young children, emphasizing the role for delayed ablation in this group. Ablation is successful for all ages. Lower recurrence occurs when electroanatomic mapping techniques are used.

Atrial Tachyarrhythmias and the Cox‐maze Procedure in Congenital Heart Disease

INTRODUCTION Atrial tachyarrhythmias, particularly atrial flutter and fibrillation, are commonly associated with congenital heart disease and are a major cause of morbidity and mortality. The Cox-maze procedure, introduced by Dr. James Cox in 1987, is effective at controlling atrial fibrillation in structurally normal hearts. Though the Cox-maze procedure has been used for atrial tachyarrhythmias in patients with congenital heart disease, few studies have looked at its effectiveness. METHODS A retrospective chart review was performed on 24 patients with congenital heart disease who underwent the Cox-maze procedure at the Medical College of Wisconsin from 2004 through 2010. RESULTS Mean age at time of Cox-maze procedure for the cohort was 40.9 years (range, 14 to 66 years). The most common congenital heart diseases among the patients included tetralogy of Fallot (n = 8) and atrioventricular septal defect (n = 4). All patients had concomitant cardiac procedures with the most common being right ventricular outflow tract reconstruction (n = 10), tricuspid valve repair (n = 8), and atrial septal defect repair (n = 7). Prior to the Cox-maze procedure, arrhythmias consisted of atrial flutter or intratrial reentrant tachycardia (n = 19) and atrial fibrillation (n = 5). There were three early postoperative deaths and one late postoperative death. Follow-up was available for 19 of 21 (90%) survivors with a mean length to follow-up from Cox-maze procedure of 2.8 years (range, 0.14-5.7 years). At last follow-up, 14 (74%) of the survivors remained arrhythmia-free. CONCLUSIONS In patients with congenital heart disease and atrial tachyarrhythmias, the majority were rendered arrhythmia-free by the Cox-maze procedure.

Development and impact of arrhythmias after the Norwood procedure: A report from the Pediatric Heart Network

Objectives: The study objective was to determine the predictors of new‐onset arrhythmia among infants with single‐ventricle anomalies during the post‐Norwood hospitalization and the association of those arrhythmias with postoperative outcomes (ventilator time and length of stay) and interstage mortality. Methods: After excluding patients with preoperative arrhythmias, we used data from the Pediatric Heart Network Single Ventricle Reconstruction Trial to identify risk factors for tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, junctional ectopic tachycardia, and ventricular tachycardia) and atrioventricular block (second or third degree) among 544 eligible patients. We then determined the association of arrhythmia with outcomes during the post‐Norwood hospitalization and interstage period, adjusting for identified risk factors and previously published factors. Results: Tachyarrhythmias were noted in 20% of subjects, and atrioventricular block was noted in 4% of subjects. Potentially significant risk factors for tachyarrhythmia included the presence of modified Blalock–Taussig shunt (P = .08) and age at Norwood (P = .07, with risk decreasing each day at age 8‐20 days); the only significant risk factor for atrioventricular block was undergoing a concomitant procedure at the time of the Norwood (P = .001), with the greatest risk being in those undergoing a tricuspid valve procedure. Both tachyarrhythmias and atrioventricular block were associated with longer ventilation time and length of stay (P < .001 for all analyses). Tachyarrhythmias were not associated with interstage mortality; atrioventricular block was associated with mortality among those without a pacemaker in the unadjusted analysis (hazard ratio, 2.3; P = .02), but not after adding covariates. Conclusions: Tachyarrhythmias are common after the Norwood procedure, but atrioventricular block may portend a greater risk for interstage mortality.

Lacosamide-induced atrial tachycardia in a child with hypoplastic left-heart syndrome: the importance of assessing additional proarrhythmic risks.

Antiepileptic medications have been reported to cause disturbances in cardiac conduction. Lacosamide decreases seizure burden by modulating sodium channels. Although it has been demonstrated to have few side effects, there have been reports of clinically significant cardiac conduction disturbances. We report the case of a child with hypoplastic left-heart syndrome and well-controlled multifocal atrial tachycardia who developed haemodynamically significant atrial tachycardia after receiving two doses of lacosamide.

Transplacental Treatment of Fetal Tachycardia: A Systematic Review and Meta-analysis

Multiple transplacental medications can be used to treat fetal tachycardia. We sought to perform a systematic review and meta‐analysis to determine whether digoxin, flecainide, or sotalol was the most efficacious therapy for converting fetal tachycardia to sinus rhythm.

Rare Cause of Infranodal Block

The patient exhibits multiple features suggestive of Timothy syndrome, which is a multisystem autosomal-dominant condition with findings that include prolonged QT interval, hand and foot abnormalities, dysmorphic facial features, and mental retardation. A 2:1 infranodal atrioventricular block may occasionally be seen in the setting of severely prolonged QT interval. Functional nature of atrioventricular block is demonstrated by resumption of 1:1 conduction with changes in heart rate.

Current Management of Focal Atrial Tachycardia in ChildrenCLINICAL PERSPECTIVE: A Multicenter Experience

Background—Focal atrial tachycardia (FAT) is an uncommon cause of supraventricular tachycardia in children. Incessant FAT can lead to tachycardia-induced cardiomyopathy. There is limited information regarding the clinical course and management of FAT. This study characterizes current management strategies for FAT in children including the prevalence of spontaneous resolution and the role of catheter ablation. Methods and Results—This is a retrospective chart review of pediatric patients with FAT managed between January 2000 and November 2010 at 10 pediatric centers. There were 249 patients with a median age at diagnosis of 7.2 (95% confidence interval, 5.8–10.4) years. Cardiomyopathy was observed in 28%. Resolution of FAT occurred in 89%, including spontaneous resolution without catheter ablation in 34%. Antiarrhythmic medications were used for initial therapy in 154 patients with control of FAT in 72%. Among first-line medications, &bgr;-blockers were the most common (53%) and effective (42%). Catheter ablation was successful in 80% of patients. FAT recurrence was less common with electroanatomic mapping compared with conventional mapping techniques (16% versus 35%; P=0.02). Patients were followed for a median of 2.1 (95% confidence interval, 1.8–2.6) years. Conclusions—FAT is managed successfully in most children. Current approaches are variable. Many patients have control of FAT with medications; however, catheter ablation is used for most patients. Spontaneous resolution is common for young children, emphasizing the role for delayed ablation in this group. Ablation is successful for all ages. Lower recurrence occurs when electroanatomic mapping techniques are used.

Current Management of Focal Atrial Tachycardia in ChildrenCLINICAL PERSPECTIVE

Background—Focal atrial tachycardia (FAT) is an uncommon cause of supraventricular tachycardia in children. Incessant FAT can lead to tachycardia-induced cardiomyopathy. There is limited information regarding the clinical course and management of FAT. This study characterizes current management strategies for FAT in children including the prevalence of spontaneous resolution and the role of catheter ablation. Methods and Results—This is a retrospective chart review of pediatric patients with FAT managed between January 2000 and November 2010 at 10 pediatric centers. There were 249 patients with a median age at diagnosis of 7.2 (95% confidence interval, 5.8–10.4) years. Cardiomyopathy was observed in 28%. Resolution of FAT occurred in 89%, including spontaneous resolution without catheter ablation in 34%. Antiarrhythmic medications were used for initial therapy in 154 patients with control of FAT in 72%. Among first-line medications, &bgr;-blockers were the most common (53%) and effective (42%). Catheter ablation was successful in 80% of patients. FAT recurrence was less common with electroanatomic mapping compared with conventional mapping techniques (16% versus 35%; P=0.02). Patients were followed for a median of 2.1 (95% confidence interval, 1.8–2.6) years. Conclusions—FAT is managed successfully in most children. Current approaches are variable. Many patients have control of FAT with medications; however, catheter ablation is used for most patients. Spontaneous resolution is common for young children, emphasizing the role for delayed ablation in this group. Ablation is successful for all ages. Lower recurrence occurs when electroanatomic mapping techniques are used.