Pharmacological Management of Cardiac Arrhythmias in the Fetal and Neonatal Periods: A Scientific Statement From the American Heart Association: Endorsed by the Pediatric & Congenital Electrophysiology Society (PACES).

Disorders of the cardiac rhythm may occur in both the fetus and neonate. Because of the immature myocardium, the hemodynamic consequences of either bradyarrhythmias or tachyarrhythmias may be far more significant than in mature physiological states. Treatment options are limited in the fetus and neonate because of limited vascular access, patient size, and the significant risk/benefit ratio of any intervention. In addition, exposure of the fetus or neonate to either persistent arrhythmias or antiarrhythmic medications may have yet-to-be-determined long-term developmental consequences. This scientific statement discusses the mechanism of arrhythmias, pharmacological treatment options, and distinct aspects of pharmacokinetics for the fetus and neonate. From the available current data, subjects of apparent consistency/consensus are presented, as well as future directions for research in terms of aspects of care for which evidence has not been established.

Ectopic Atrial Tachycardia in Infants Following Congenital Heart Disease Surgery.

Although ectopic atrial tachycardia (EAT) is common following surgery for congenital heart disease (CHD), there are limited data regarding this arrhythmia. This study assessed risk factors and outcomes for patients less than one year of age with post-operative EAT. This was a retrospective analysis of infants undergoing CHD surgery from 2007 to 2020. Patients and surgeries with EAT were compared to controls without EAT. Out of 5372 infant CHD surgeries, EAT developed in 129 (2.5%). Compared to controls, the EAT cohort was younger (median 7 vs 85 days, p < 0.01), weighed less at time of surgery (3.3 vs 4.2 kg, p < 0.01), and was more likely to have DiGeorge syndrome (7.7% vs 3.0%, p < 0.01). Multivariate analysis revealed total anomalous venous connection (TAPVC) repair (odds ratio [OR] 2.8; 95% confidence interval 1.5-5.2), DiGeorge syndrome (OR 2.4; 1.1-5.2), Society of Thoracic Surgeons-European Association for Cardio-Thoracic surgery (STAT) category ≥ 4 (OR 2.1; 1.0-4.4), and longer cardiopulmonary bypass times (OR 1.1; 1.0-1.2) as independent risk factors for EAT. The onset of EAT occurred a median of 9 days (IQR 5-14 days) after CHD surgery. Antiarrhythmic treatment was initiated in 109/129 patients (84%) with propranolol (71%) and amiodarone (24%) the most commonly used medications. Although 15 (11.6%) patients did not survive to hospital discharge, EAT was not directly implicated in any deaths. EAT occurred after 2.5% of infant CHD surgeries. In addition to TAPVC repair, longer and more complex surgeries were associated with an increased the risk for the development of post-operative EAT.

Sudden cardiac death in congenital heart disease.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms.

Third Trimester Fetal Heart Rates in Antibody-Mediated Complete Heart Block Predict Need for Neonatal Pacemaker Placement.

Congenital complete heart block (CCHB) affects 1 in 20,000 newborns. This study evaluates fetal and neonatal risk factors predictive of neonatal pacemaker placement in antibody-mediated complete heart block. The Children's Hospital Los Angeles institutional fetal, pacemaker, and medical record databases were queried for confirmed SSA/SSB cases of CCHB between January 2004 and July 2019. Cases excluded were those with a diagnosis beyond the neonatal period, diagnosis of a channelopathy, or if maternal antibody status was unknown. We recorded the gestational age (GA), birth weight (BW), fetal heart rates (FHRs) of the last echocardiogram before delivery, specific neonatal ECG and echocardiogram findings, age at pacemaker placement, and mortality. Of 43 neonates identified with CCHB, 27 had confirmed maternal antibody exposure. Variables associated with neonatal pacemaker implantation were FHRs < 50 bpm (p = 0.005), neonatal heart rates < 52 bpm (p = 0.015), and neonatal left ventricular fractional shortening (FS) percentages < 34% (p = 0.03). On multivariate analysis, FHR remained significant (p = 0.03) and demonstrated an increased risk of neonatal pacemaker placement by an odds ratio of 12.5 (95% CI 1.3-116, p = 0.05). The median GA at which the FHR was obtained was 34 weeks (IQR 26-35 weeks). Neonatal pacemaker placement was highly associated with a FHR < 50 bpm, neonatal HR < 52 bpm, and neonatal FS < 34%. FHRs at 34 weeks GA (IQR 26-35 weeks) correlated well with postnatal heart rates and were predictive of neonatal pacemaker placement.

Variations in cardiac implantable electronic device surveillance and ancillary testing in the paediatric and congenital heart disease population: an international multi-centre survey from the Paediatric and Congenital Electrophysiology Society.

BACKGROUND: Expert guidance from scientific societies and regulatory agencies recommend a framework of principles for frequency of in-person evaluations and remote monitoring for patients with cardiac implantable electronic devices. However, there are limited data regarding adherence to recommendations among paediatric electrophysiologists, and there are no data regarding cardiac implantable electronic device-related ancillary testing. METHODS: To assess current clinical practices for cardiac implantable electronic device in-person evaluation, remote monitoring, and cardiac implantable electronic device-related ancillary testing, the Paediatric and Congenital Electrophysiology Society members were surveyed. The main outcome measures were variations in frequency of in person evaluation, frequency of remote monitoring, and cardiac implantable electronic device-related ancillary testing. RESULTS: All respondents performed in-person evaluation at least once a year, but <50% of respondents performed an in-person evaluation within 2 weeks of cardiac implantable electronic device implantation. Remote monitoring was performed every 3 months for pacemakers and implantable cardioverter defibrillators by 71 and 75% respondents, respectively. Follow-up echocardiography was performed every 2-3 years by 53% respondents for patients with >50% ventricular pacing. Majority of respondents (75%) did not perform either an exercise stress test or ambulatory Holter monitoring or chest X-ray (65%) after cardiac implantable electronic device implantation. CONCLUSION: This survey identified significant practice variations in cardiac implantable electronic device in- person evaluation, remote monitoring, and ancillary testing practices among paediatric electrophysiologists. Cardiac implantable electronic device management may be optimised by development of a paediatric-specific guidelines for follow-up and ancillary testing.

Utility of Surveillance Ambulatory Rhythm Monitoring in the Pediatric Fontan Population.

Our institution established a Fontan surveillance plan, which included ambulatory rhythm monitoring (ARM) at 6, 10, 13, 16 and 19 years old, for early detection of Fontan-associated complications. We conducted a retrospective chart review of Fontan patients followed at our institution 2014-2018 to determine the utility of surveillance ARMs. 139 ARMs from 83 patients were included. ARMs with supraventricular tachycardia, sinus node dysfunction, accelerated junctional rhythm, > 1st degree atrioventricular block, and complex ventricular ectopy were classified as positive for arrhythmia. Arrhythmias were occult if detected on surveillance ARM. The ARM indication was surveillance in 78 (56%) and clinically indicated in 61 (44%). 52 (37%) ARMs in 27 (33%) patients had an arrhythmia. There was no difference in the age of patients with and without arrhythmias [median 10.9 (6.5, 17.1 years) vs. 8.8 (7, 13.6 years), p = 0.5]. Clinically indicated ARMs more frequently demonstrated arrhythmias than surveillance ARMs (52% vs. 26%, p < 0.01). Compared to patients without arrhythmias, those with arrhythmias were more likely to be female (48% vs. 23%, p = 0.02), have a single right ventricle (46% vs. 19%, p < 0.01) and longer QRS duration on ECG [100 (91, 116 ms) vs. 94 (84, 104 ms), p = 0.046]. Patients with occult arrhythmias were less likely to have moderate to severe atrioventricular valvar regurgitation (0% vs. 46%; p = 0.04) or ventricular dysfunction (0% vs. 46%; p = 0.04) than those with clinical arrhythmia(s). Arrhythmia findings resulted in change in management for 16/52 (31%) ARMs. The findings suggest the frequent presence of arrhythmias on periodic ARMs in patients following the Fontan procedure regardless of symptomatic status.

Characteristics of antegrade-only accessory pathways in children and adolescents.

There is minimal data regarding antegrade-only accessory pathways in young patients. Given evolving recommendations and treatments, retrospective analysis of the clinical and electrophysiologic properties of antegrade-only pathways in patients <21 years old was performed, with subsequent comparison of electrophysiology properties to age-matched controls with bidirectional pathways. Of 522 consecutive young patients with ventricular pre-excitation referred for electrophysiology study, 33 (6.3%) had antegrade-only accessory pathways. Indications included palpitations (47%), chest pain (25%), and syncope (22%). The shortest value for either the accessory pathway effective refractory period or the pre-excited R-R interval was taken for each patient, with the median of the antegrade-only group significantly greater than shortest values for the bidirectional group (310 [280-360] ms versus 270 [240-302] ms, p < 0.001). However, the prevalence of pathways with high-risk properties (effective refractory period or shortest pre-excited R-R interval <250 ms) was similar in both study patients and controls (13% versus 21%) (p = 0.55). Sixteen patients had a single antegrade-only accessory pathway and no inducible arrhythmia. Six patients had Mahaim fibres, all right anterolateral with inducible antidromic reciprocating tachycardia. However, 11 patients with antegrade-only accessory pathways and 3 with Mahaim fibres had inducible tachycardia due to a second substrate recognised at electrophysiology study. These included concealed accessory pathways (7), bidirectional accessory pathways (5), and atrioventricular node re-entry (2). Antegrade-only accessory pathways require comprehensive electrophysiology evaluation as confounding factors such as high-risk conduction properties or inducible Supraventricular Tachycardia (SVT) due to a second substrate of tachycardia are often present.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

A prospective 5-year study of exercise performance following Melody valve implant.

BACKGROUND: The long-term benefits of Melody valve implant for right ventricular outflow tract conduit obstruction or insufficiency on exercise capacity are undefined. METHODS: As part of the Melody valve clinical trial, 136 patients with congenital heart disease underwent serial cardiopulmonary exercise testing prior to, 6 months after, and annually for up to 5 years postimplant. RESULTS: Mean age at Melody valve implantation was 22.4 ± 0.9 years (range 7-53 years). The 95 patients who completed the study protocol provide the basis of this report. An initial improvement in % predicted workload was present at 6 months postimplant; however, at the final (5 year) follow-up, sustained or further improvements in workload were not demonstrated for the entire cohort compared to baseline. By subgroup analysis, age <17 years at implant and pulmonary regurgitation as the primary lesion were variables associated with sustained improvement in exercise performance. There were sustained improvements in the ventilatory equivalents for O2 (minute ventilation/O2 intake, P = .01) and CO2 (minute ventilation/CO2 output, P < .01) at the ventilatory anaerobic threshold at the study conclusion. Improvements in forced vital capacity were also observed during the study but not sustained at the final follow-up. CONCLUSIONS: A cautious appraisal of the cardiovascular benefits of Melody valve implant on sustained improvements in exercise performance appears warranted. Although the observed changes in pulmonary function suggest improved restrictive lung physiology and more efficient gas exchange, after an initial increase in % predicted performance, neither sustained nor further improvements in exercise performance were observed, except in specific patient subgroups.

A comparison of oral flecainide and amiodarone for the treatment of recurrent supraventricular tachycardia in children.

BACKGROUND: Supraventricular tachycardia (SVT) in children can be difficult to treat when first-line therapies (beta-blockade or digoxin) are not effective. Both flecainide and amiodarone are used as second-line therapies. We sought to compare the efficacy and safety of flecainide and amiodarone in pediatric patients with recurrent SVT. METHODS: Pediatric patients treated with oral flecainide or oral amiodarone for SVT between 2006 and 2015 were studied. Tachycardia mechanisms included orthodromic reciprocating tachycardia (ORT), intra-atrial reentrant tachycardia (IART), and ectopic atrial tachycardia (EAT). Outcomes were classified as full success, partial success (requiring additional intervention), or failure. RESULTS: Seventy-four patients were included (median age 46 days, range 1 day to 19 years). Flecainide was used in 47 patients and amiodarone in 27 patients. Full success was achieved in 68% and 59%, respectively (P = 0.28). Partial success was achieved in 13% and 19%, respectively (P = 0.12). Treatment failed in 19% and 22%, respectively (P = 0.97). Ten crossover patients received the second medication after the first failed. Of five amiodarone-to-flecainide crossovers, four achieved success on flecainide alone. Of five flecainide-to-amiodarone crossovers, two achieved success. Minor adverse events occurred in 9% of flecainide and 22% of amiodarone patients (P = 0.16). No significant differences were seen by arrhythmia subtype (36 EAT, 28 ORT, 10 IART), congenital heart disease (n = 38), or age group (56 infants). CONCLUSIONS: Oral flecainide and amiodarone achieved meaningful arrhythmia control in 81% and 78% of pediatric patients with recurrent SVT, respectively. Those who failed amiodarone had encouraging outcomes when changed to flecainide.

Home use of a compact, 12­lead ECG recording system for newborns.

BACKGROUND: An easy-to-operate ECG recorder should be useful for newborn screening for heart conditions, by health care workers - or parents. We developed a one-piece electrode strip and a compact, 12­lead ECG recorder for newborns. METHOD: We enrolled 2582 newborns in a trial to assess abilities of parents to record a 12­lead ECG on their infants (2-4 weeks-old). Newborns were randomized to recordings by parents (1290) or our staff (1292 controls). Educational backgrounds of parents varied, including 64% with no more than a high school diploma. RESULTS: For newborns randomized to parent recorded ECGs, 94% of parents completed a 10-minute recording. However, 42.6% asked for verbal help, and 12.7% needed physical help. ECG quality was the same for recordings by parents versus staff. CONCLUSIONS: By use of a one-piece electrode strip and a compact recorder, 87% of parents recorded diagnostic quality ECGs on their newborn infants, with minimal assistance.

Return to play in the athlete with cardiac disease: who decides and what is the protocol?

Improved public awareness and advances in medical diagnostics have resulted in the development of criteria to determine eligibility or disqualification for the athlete with cardiovascular abnormalities. Simultaneously, protocols have been developed for athletes with concussion or orthopaedic injuries to guide team physicians and consultants in return-to-play decisions. However, there are currently inadequate data to allow the development of such protocols for athletes with cardiovascular abnormalities who have undergone treatment. Further complicating the decision process is the designation of the team physician as the ultimate authority in making return-to-play decisions - where the team physician often is an employee of the team and supports the team's goal and players as well as the individual athlete. This review will discuss the ethical dilemma of the team physician and the role of the cardiovascular consultant. Following this, current data and practices regarding return to play will be discussed for the following conditions or diagnoses: following catheter ablation for supraventricular tachycardia; following pacemaker or implantable cardioverter-defibrillator implantation; unexplained syncope; and the athlete with a genetic mutation in the absence of any phenotype of associated disease. These recommendations will undoubtedly continue to evolve and improve and should be considered at this time as a point of departure.

Comparison of formulas for calculation of the corrected QT interval in infants and young children.

OBJECTIVE: To compare 4 heart rate correction formulas for calculation of the rate corrected QT (QTc) interval among infants and young children. STUDY DESIGN: R-R and QT intervals were measured from digital electrocardiograms. QTc were calculated with the Bazett, Fridericia, Hodges, and Framingham formulas. QTc vs R-R graphs were plotted, and slopes of the regression lines compared. Slopes of QTc-R-R regression lines close to zero indicate consistent QT corrections over the range of heart rates. RESULTS: We reviewed electrocardiograms from 702 children, with 233 (33%) <1 year of age and 567 (81%) <2 years. The average heart rate was 122 ± 20 bpm (median 121 bpm). The slopes of the QTc-R-R regression lines for the 4 correction formulas were -0.019 (Bazett); 0.1028 (Fridericia); -0.1241 (Hodges); and 0.2748 (Framingham). With the Bazett formula, a QTc >460 ms was 2 SDs above the mean, compared with "prolonged" QTc values of 414, 443, and 353 ms for the Fridericia, Hodges, and Framingham formulas, respectively. CONCLUSIONS: The Bazett formula calculated the most consistent QTc; 460 ms is the best threshold for prolonged QTc. The study supports continued use of the Bazett formula for infants and children and differs from the use of the Fridericia correction during clinical trials of new medications.

A Comparison of AV Nodal Reentrant Tachycardia in Young Children and Adolescents: Electrophysiology, Ablation, and Outcomes.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) typically occurs in adolescents and adults with limited data regarding AVNRT in young children. METHODS: All patients with AVNRT who underwent electrophysiology study and ablation between 2005 and 2012 were retrospectively studied. Patients were stratified by age <10 years (young AVNRT) or ≥10 years (older AVNRT). Young AVNRT patients were also compared to age-matched patients with orthodromic reentrant tachycardia (ORT). RESULTS: A total of 275 studies in 272 patients were evaluated including 38 young AVNRT patients (7.7 ± 1.5 years) and 202 older AVNRT patients (14.9 ± 2.1 years). An atrial-His jump ≥50 ms was demonstrated in 56% of young and 64% of older patients. Slow pathway modification was attempted in all but one older patient with acute ablation success achieved in all. RF ablation was the primary ablation modality with cryoablation used in 10 patients (three young and seven older). Recurrences were rare (zero young and three older patients) despite residual AVNRT echo beats postablation in 34% of young and 40% of older patients. One older AVNRT patient (0.5%) required a pacemaker for heart block while no complications occurred in the young patients. Electrophysiologic parameters were comparable to the 35 age-matched young ORT group (7.7 ±1.7 years) in whom supraventricular tachycardia was more inducible. CONCUSIONS: Slow pathway modification for AVNRT in children resulted in high success and low complication rates, regardless of age. Recurrence of tachycardia was infrequent despite persistence of AVNRT echo beats in 34-40% of patients following slow pathway modification.

Current practices in the monitoring of cardiac rhythm devices in pediatrics and congenital heart disease.

Although guidelines for routine follow-up of pacemakers and implantable cardioverter defibrillators (ICDs) are available for adults, minimal data supports their appropriateness in pediatrics and congenital heart disease. This study aimed to define current practices of cardiac rhythm device (CRD) follow-up among pediatric electrophysiologists. Pediatric and Congenital EP Society (PACES) members were surveyed regarding frequency of CRD in-person follow-up as well as transtelephonic monitoring (TTM) and remote monitoring (RM) practices. If home monitoring was used, the effect on in-person follow-up was also evaluated. A total of 106 PACES members responded to the survey. Uncomplicated pacemaker and ICD patients were both followed in-person at a median interval of 6 months (range 1-12 months). TTM was utilized by 67 % of responders (median interval 3 months; range 1-6 months), while RM was used by 87 % for pacemakers (median interval 3 months; range 1-6 months) and 92 % for ICDs (median interval 3 months; range 2 weeks-6 months). When TTM was used, 21 % of responders reduced their frequency of pacemaker clinic visits. In comparison, RM reduced the frequency of clinic visits for pacemakers and ICDs in 32 and 31 % of responders, respectively. Patient age was an independent factor in determining CRD follow-up for 49 % of responders. While CRD follow-up by pediatric electrophysiologists in general follows adult guidelines, individual practices widely vary. In contrast to published recommendations in adults, TTM and RM utilization does not reduce the frequency of in-person visits for the majority of pediatric electrophysiologists.

Genetic variants for long QT syndrome among infants and children from a statewide newborn hearing screening program cohort.

OBJECTIVES: Autosomal recessive long QT syndrome (LQTS), or Jervell and Lange-Nielsen syndrome (JLNS), can be associated with sensorineural hearing loss. We aimed to explore newborn hearing screening combined with electrocardiograms (ECGs) for early JLNS detection. STUDY DESIGN: In California, we conducted statewide, prospective ECG screening of children ≤ 6 years of age with unilateral or bilateral, severe or profound, sensorineural or mixed hearing loss. Families were identified through newborn hearing screening and interviewed about medical and family histories. Twelve-lead ECGs were obtained. Those with positive histories or heart rate corrected QT (QTc) intervals ≥ 450 ms had repeat ECGs. DNA sequencing of 12 LQTS genes was performed for repeat QTc intervals ≥ 450 ms. RESULTS: We screened 707 subjects by ECGs (number screened/number of responses = 91%; number of responses/number of families who were mailed invitations = 54%). Of these, 73 had repeat ECGs, and 19 underwent gene testing. No subject had homozygous or compound heterozygous LQTS mutations, as in JLNS. However, 3 individuals (with QTc intervals of 472, 457, and 456 ms, respectively) were heterozygous for variants that cause truncation or missplicing: 2 in KCNQ1 (c.1343dupC or p.Glu449Argfs*14; c.1590+1G>A or p.Glu530sp) and 1 in SCN5A (c.5872C>T or p.Arg1958*). CONCLUSIONS: In contrast to reports of JLNS in up to 4% of children with sensorineural hearing loss, we found no examples of JLNS. Because the 3 variants identified were unrelated to hearing, they likely represent the prevalence of potential LQTS mutations in the general population. Further studies are needed to define consequences of such mutations and assess the overall prevalence.

Attention-deficit/hyperactivity disorder, stimulant therapy, and the patient with congenital heart disease: evidence and reason.

Attention-deficit/hyperactivity disorder (ADHD) is the most common neurobehavioral disorder of childhood, and congenital heart disease (CHD) is the most common form of birth defect. Children with CHD are at increased risk for neurodevelopmental disorders such as ADHD. Stimulant medications, specifically methylphenidates and amphetamines, are frequently prescribed and effective in reducing the symptoms of ADHD. Despite their efficacy and long history of use, the safety of these medications has recently come into question due to isolated reports describing sudden unexplained death of children undergoing treatment. This review summarizes the current literature on the cardiovascular risks associated with the use of pharmacologic therapy for ADHD, with an emphasis on patients who had CHD.