The effectiveness of implantable cardioverter-defibrillator (ICD) therapy for the management of catecholaminergic polymorphic ventricular tachycardia (VT) in young patients is not known. ICD ...discharges are not always effective and inappropriate discharges are common, both resulting in morbidity and mortality.
This is a multicenter, retrospective review of young patients with catecholaminergic polymorphic VT and ICDs from 5 centers. ICD discharges were evaluated to determine arrhythmia mechanism, appropriateness, efficacy of therapy, and complications. A total of 24 patients were included. Median (interquartile range) ages at onset of catecholaminergic polymorphic VT symptoms and ICD implant were 10.6 (5.0-13.8) years and 13.7 (10.7-16.3) years, respectively. Fourteen patients received 140 shocks. Ten patients (42%) experienced 75 appropriate shocks and 11 patients (46%) received 65 inappropriate shocks. On actuarial analysis, freedom from appropriate shock at 1 year after ICD implant was 75%. Of appropriate shocks, only 43 (57%) demonstrated successful primary termination. All successful appropriate ICD discharges were for ventricular fibrillation. No episodes of polymorphic VT or bidirectional VT demonstrated successful primary termination. The adjusted mean (95% confidence interval) cycle length of successful discharges was significantly shorter than unsuccessful discharges (168 152-184 ms versus 245 229-262 ms; adjusted P=0.002). Electrical storm occurred in 29% (4/14) and induction of more malignant ventricular arrhythmias in 36% (5/14). There were no deaths.
ICD efficacy in catecholaminergic polymorphic VT depends on arrhythmia mechanism. Episodes of ventricular fibrillation were uniformly successfully treated, whereas polymorphic and bidirectional VT did not demonstrate successful primary termination. Inappropriate shocks, electrical storm, and ICD complications were common.
Insights on Atrial Fibrillation in Congenital Heart Disease Ebrahim, Mohammad A.; Escudero, Carolina A.; Kantoch, Michal J. ...
Canadian journal of cardiology,
November 2018, 2018-11-00, 20181101, Letnik:
34, Številka:
11
Journal Article
Recenzirano
Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias being the most common long-term complication. In recent reports, atrial fibrillation (AF) ...tended to be the most common form of arrhythmias among groups of patients with adult CHD (ACHD) older than 50 years of age. When compared with their adult counterparts without CHD, AF in patients with ACHD has been characterized by a higher incidence and prevalence, younger age of onset, and a greater risk of progression to persistent AF. Risk factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension, and multiple cardiac surgeries. Data on management options such as optimal antiarrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation are limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology, and management options for AF in this complex patient population.
Chez les patients atteints de cardiopathie congénitale (CC), qui peuvent survivre jusqu’à un âge adulte avancé, les arythmies auriculaires constituent les complications à long terme les plus fréquentes. Dans les études publiées récemment, la fibrillation auriculaire (FA) était en général la forme d’arythmie la plus répandue chez les patients atteints de cardiopathie congénitale adulte (CCA) âgés de plus de 50 ans. Comparativement à celle qui touche les adultes non atteints de CC, la FA chez les patients atteints de CCA est caractérisée par une incidence et une prévalence supérieures, un âge de survenue plus précoce et un risque plus élevé d’évolution vers une FA persistante. Les facteurs de risque d’apparition de la FA ne sont pas bien connus, mais comprennent le vieillissement, la dilatation auriculaire gauche, l’hypertension systémique et le fait d’avoir subi plusieurs chirurgies cardiaques. Les données sur les options de prise en charge comme le traitement optimal par un médicament antiarythmique, les indications des anticoagulants et l’efficacité et l’innocuité de l’ablation par cathéter sont peu nombreuses. Il est impératif d’approfondir les recherches pour explorer les stratégies de prise en charge, de prévention et de surveillance destinées à la population croissante de patients atteints de CCA et de FA. Ce rapport passe en revue les données les plus récentes sur l’épidémiologie, la physiopathologie et les options de prise en charge de la FA dans cette population de patients complexes.
Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high ...medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published.
To describe the presentation and clinical course of PJRT in children.
This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010.
The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients.
PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.
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.
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, β-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.
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.
Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with ...HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients’ weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18–50, mean 29.1) was present in 140 children aged 2–19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (
p
= 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.
Abstract Background There are few reports of pediatric studies of atrial fibrillation (AF). We sought to describe the clinical characteristics, management strategies, and recurrence rates and to ...identify predictors of AF recurrence in a contemporary pediatric population. Methods A retrospective review was performed of patients ≤ 18 years with lone AF who were seen at 4 pediatric institutions from 1996-2011. Patients with AF in the setting of thyroid disease, ventricular pre-excitation, coexisting congenital heart disease, or a history of cardiac surgery were excluded. Demographics, clinical presentation, investigations, treatment, and follow-up were analyzed. Results Forty-two patients were diagnosed with a first episode of lone AF, and 4 of these cases were later classified as persistent AF. Thirty-one (74%) were male patients, median age was 15.3 years, and median (interquartile range IQR) duration of AF episode was 12 (IQR, 7-24) hours. AF recurred in 39% (15 of 38) of patients. The Kaplan-Meier median time to estimated recurrence was 19 months. By univariate analysis, initial AF episode duration was associated with a higher risk of recurrence (hazard ratio HR, 1.01; 95% confidence interval CI, 1-1.02; P = 0.034). Sex, age, family history, size of the left atrium, and history of cardioversion were not associated with recurrence. Recurrence with another supraventricular tachyarrhythmia (SVT) was observed in 6 of 38 (16%) patients, and 12 patients underwent electrophysiology (EP) study, with 6 patients receiving ablation. Conclusions Our reported rate of recurrence of 39% is important when counseling pediatric patients and their parents on the expected course and treatment goals.
Several different mechanisms are responsible for paroxysmal supraventricular tachycardia in children. Different forms of tachycardia occur at different age. Atrio-ventricular reentry tachycardia ...results from the presence of congenital atrio-ventricular bypass tracts and is frequently encountered at all ages. Infants may present with ectopic atrial tachycardia or atrial flutter. Atrio-ventricular node reentry tachycardia becomes more frequent in adolescence. Atrial scarring resulting from open heart surgery predisposes to complex intra-atrial reentry. Certain forms of congenital and acquired heart disease are associated with specific types of arrhythmia. Many children with paroxysmal supraventricular tachycardia do not require any therapy. The decision to proceed with treatment should be based on the frequency and severity of symptoms and on the effect of arrhythmia on the quality of life. Infants require medical treatment because of the difficulty to recognize symptoms of tachycardia and a risk of heart failure. Patients with Wolff-Parkinson-White syndrome as well as those with significant heart disease are at risk of sudden death. Syncope in children with paroxysmal tachycardia may indicate a severe fall in cardiac output from extremely rapid heart rate. Patients with potentially life-threatening arrhythmia should not participate in competitive physical activities. Treatment options have undergone significant evolution over the past decade. Indications for the use of specific antiarrhythmic medications have been refined. Contemporary catheter ablation procedures employ different forms of energy allowing for safe and effective procedures. Catheter ablation is the treatment of choice for symptomatic paroxysmal tachycardia in school children and in some infants who failed medical treatment. Surgery is the preferred treatment in few selected cases. The goal of this review is to present the state of the art approach to the diagnosis and management of paroxysmal supraventricular tachycardia in infants, children and adolescents.
The study was designed to assess atrio-ventricular (AV) conduction with non-invasive methods at least 1 year after radiofrequency ablation (RFA) of the slow pathway for AV node reentry tachycardia.
...Medical records of all patients who underwent RFA before their 18th birthday were reviewed. Patients were brought back for clinical evaluation, an electrocardiogram, an exercise stress test, and ambulatory Holter monitoring. Radiofrequency ablation of the slow pathway above the ostium of the coronary sinus was done in 106 children. No procedure resulted in high degree AV block. Follow-up evaluation was possible in 67 patients (63% of the total cohort) who were brought back to the clinic 1-13.7 years, mean 4.7+/-3.0 years after the procedure. Dizzy spells were reported by 36% of examined patients and 2 patients reported syncope. PR intervals were normal in all but two patients when compared with published normal values. One patient presented with persistent, post-procedural first-degree AV block and another developed new onset, symptomatic second degree AV block 2 years after the procedure and required pacemaker implantation.
Non-invasive testing showed normal PR intervals in a cohort of patients who underwent RFA of the slow pathway in childhood or adolescence. Late AV block occurred in one child. Clinical evaluation more than a year after the procedure is warranted in symptomatic patients.
Fluconazole is an antifungal medication that has been reported to cause prolongation of the QT interval and Torsades de Pointes (TdP) ventricular tachycardia in adults. We describe the case of an ...11-year-old child treated with Fluconazole who developed ventricular arrhythmia culminating in TdP. We discuss the possible roles played by genetic and environmental factors in this child’s rhythm disturbances. After briefly summarizing similar cases from the adult literature, we outline the putative mechanism by which Fluconazole may cause arrhythmia. This case should alert pediatricians to the possible risks of Fluconazole use, especially in the presence of electrolyte abnormalities, diuretic use, therapy with other pro-arrhythmic agents, or suspicion of congenital Long-QT Syndrome.