SOS1-Related Noonan Syndrome and Sudden Cardiac Arrest in the Absence of Cardiomyopathy-An Arrhythmia Phenotype?

Noonan syndrome (NS) is a predominantly autosomal dominant condition with various cardiac and extra-cardiac manifestations. Although it has been linked with atrial arrhythmias, ventricular arrhythmias are extremely rare in the absence of underlying structural cardiac abnormalities. We report an instance of aborted sudden cardiac arrest in a 7-year-old male with a confirmed SOS1 variant and a lack of evidence to support a structural cardiac, metabolic, or infectious etiology. This is the second reported instance of sudden cardiac arrest related to ventricular fibrillation in a child with SOS1-related NS in the absence of any structural cardiac defects. Although no definitive correlation can be ascertained from a limited existing body of knowledge surrounding SOS1 and ventricular fibrillation unrelated to structural heart defects, it provokes the idea of an arrhythmia phenotype and future research is warranted to guide proper clinical treatment, monitoring, and management of such individuals.

Utility of routine follow-up defibrillation safety margin testing in young patients with epicardial implantable cardioverter-defibrillators.

BACKGROUND: Routine defibrillation threshold testing (DFT) of transvenous implantable defibrillators (ICDs) has largely been in decline. In patients with non-transvenous ICDs that utilize subcutaneous and pleural ICD leads, serial DFT testing can detect a significant number of failures. Data about the utility of follow-up defibrillation safety margin testing (DSM) testing in pediatric patients and young adults with an epicardial ICD are lacking. METHODS: Patients aged < 25 years old who underwent epicardial ICD placement at Mayo Clinic from 2014 to 2023 with at least one follow-up DSM test were included. The patients were divided into a "routine" (R) and "clinically indicated" (CI) group based on the index of clinical concern. Inadequate DSM was defined as unsuccessful defibrillation at an output of less than 10 J below the maximum output of the device. The purpose of this study was to assess the utility of follow-up DSM testing. RESULTS: An epicardial ICD system was placed in 122 patients. A total of 26 patients met inclusion criteria and underwent a total of 47 DSM follow up tests. Inadequate DSM occurred in 1/33 (3%) in the R group and 2/14 (14%) DSM tests in the CI group. The median follow-up period was 54 and 36 months for the R and CI group, respectively. CONCLUSIONS: Our data suggest that epicardial ICDs are reliable and routine follow-up DSM testing may not be necessary for all patients. DSM testing should be performed in individuals with epicardial ICD systems when there is clinical concern about lead or coil performance.

Empiric Slow Pathway Cryoablation in Symptomatic Children Without Documented Supraventricular Tachycardia.

In symptomatic children without documented supraventricular tachycardia (SVT) and non-inducible atrioventricular nodal reentry tachycardia (AVNRT) the benefit of empiric slow pathway (SP) ablation is unknown. We evaluated 62 symptomatic patients without documented SVT that underwent electrophysiology study (EPS). The purpose of this study was to determine if symptoms improved after empiric SP ablation in children without documented SVT and without inducible AVNRT. Sixty-two symptomatic patients without previously documented SVT underwent EPS; 31 (50%) had inducible AVNRT and underwent SP ablation, 20 (32%) were non-inducible and underwent empiric SP ablation, 11 (18%) were non-inducible and had no ablation. After a mean follow-up of 23 ± 18 months there was no significant difference in freedom from symptoms within the non-inducible cohort regardless of whether empiric SP ablation was performed (p = 0.135). There was a significant improvement in symptoms at follow-up after SP ablation when comparing inducible and non-inducible patients (p = 0.020). During follow-up no patients had documented SVT. Symptomatic children without documented SVT do not benefit from empiric SP ablation when AVNRT cannot be induced.

Safety and efficacy of (+)-epicatechin in subjects with Friedreich's ataxia: A phase II, open-label, prospective study.

BACKGROUND: (+)-Epicatechin (EPI) induces mitochondrial biogenesis and antioxidant metabolism in muscle fibers and neurons. We aimed to evaluate safety and efficacy of (+)-EPI in pediatric subjects with Friedreich's ataxia (FRDA). METHODS: This was a phase II, open-label, baseline-controlled single-center trial including 10 participants ages 10 to 22 with confirmed FA diagnosis. (+)-EPI was administered orally at 75 mg/d for 24 weeks, with escalation to 150 mg/d at 12 weeks for subjects not showing improvement of neuromuscular, neurological or cardiac endpoints. Neurological endpoints were change from baseline in Friedreich's Ataxia Rating Scale (FARS) and 8-m timed walk. Cardiac endpoints were changes from baseline in left ventricular (LV) structure and function by cardiac magnetic resonance imaging (MRI) and echocardiogram, changes in cardiac electrophysiology, and changes in biomarkers for heart failure and hypertrophy. RESULTS: Mean FARS/modified (m)FARS scores showed nonstatistically significant improvement by both group and individual analysis. FARS/mFARS scores improved in 5/9 subjects (56%), 8-m walk in 3/9 (33%), 9-peg hole test in 6/10 (60%). LV mass index by cardiac MRI was significantly reduced at 12 weeks (P = .045), and was improved in 7/10 (70%) subjects at 24 weeks. Mean LV ejection fraction was increased at 24 weeks (P = .008) compared to baseline. Mean maximal septal thickness by echocardiography was increased at 24 weeks (P = .031). There were no serious adverse events. CONCLUSION: (+)-EPI was well tolerated over 24 weeks at up to 150 mg/d. Improvement was observed in cardiac structure and function in subset of subjects with FRDA without statistically significant improvement in primary neurological outcomes. SYNOPSIS: A (+)-epicatechin showed improvement of cardiac function, nonsignificant reduction of FARS/mFARS scores, and sustained significant upregulation of muscle-regeneration biomarker follistatin.

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.

Placement of Reveal LINQ Device in the Left Anterior Axillary Position.

Implantable loop recorders (ILR) are utilized for long-term rhythm monitoring. Typical placement of the Medtronic Reveal LINQ along the left parasternal border may compromise the quality and/or feasibility of future imaging studies. We sought to evaluate the utility of placing an ILR in the left anterior axillary position and the impact on the quality of cardiac imaging. We reviewed patients from May 2017 to June 2018 who had placement of a Reveal LINQ device in the left anterior axillary position. Demographic, procedural, and clinical data were collected via retrospective review. Cardiac magnetic resonance imaging (MRI) studies were reviewed for image quality after ILR placement. Eight patients met inclusion criteria for this study (median age 6 years, 50% female). Six patients (75%) had an ILR placed in the operating room, while all others were placed in the electrophysiology lab. All patients demonstrated acceptable R waves for diagnostic evaluation (median = 0.85 mV, range 0.24-1.7 mV). Cardiac MRI was obtained in 7 patients following ILR placement with diagnostic image quality and no adverse events. One device was explanted 28 days after placement due to concern for possible infection. No other devices required removal or revision (median follow up duration 11 months, IQR 8-13.5). ILR placement in the left anterior axillary position can record adequate signals in pediatric patients. In addition, axillary ILR device position may allow for completion of cardiac imaging, particularly cardiac MRI, without significant artifacts which is critical for patients with congenital heart disease.

Quality of life in young patients after cone reconstruction for Ebstein anomaly.

OBJECTIVE: To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly. METHODS: We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning. RESULTS: Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed "at risk" for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time. CONCLUSION: Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.

Electrocardiographic abnormalities in elite high school athletes: comparison to adolescent hypertrophic cardiomyopathy.

BACKGROUND: In athletes, ECG changes from physiological cardiac remodelling are common but can overlap with findings from a pathological disorder. We compared ECG findings in a group of elite high school athletes to a cohort of adolescents with hypertrophic cardiomyopathy (HCM). METHODS/RESULTS: We prospectively performed 15-lead ECGs and echocardiograms in 147 elite high school athletes. Student-athlete ECGs were compared in blinded fashion to ECGs of 148 adolescents with HCM of similar age and ethnicity. Standard ECG hypertrophy criteria and established expert opinion guidelines (European Society of Cardiology, ESC and Seattle criteria) were analysed. All student-athletes had normal echocardiograms. Overall, 77/147 (52%) of student-athletes met standard ECG criteria for ventricular hypertrophy compared to 126/148 (85%) adolescents with HCM (p<0.0001). There were 112/148 (76%) adolescents with HCM who had pathological Q-waves, T-wave inversion and/or ST-segment depression compared to 1/147 (1%) athletes (p<0.0001). Most patients with HCM (84%, 124/148) had ≥1 abnormal ECG finding(s) according to Seattle criteria, compared to 1% of student-athletes (2/147). Similarly, 130/148 (88%) patients with HCM met group-2 ESC criteria (abnormal), compared to 36/147 (24%) student-athletes (p<0.0001). CONCLUSIONS: Over 50% of elite high school athletes with echocardiographically confirmed normal hearts satisfied standard voltage criteria for ventricular hypertrophy. Pathological Q-waves, T-wave inversion or ST-segment depression were most helpful in distinguishing adolescents with HCM from normals. Both ESC and Seattle criteria successfully stratified the student-athlete and HCM cohorts, however each had a false-negative rate >10% for the HCM cohort. The Seattle criteria demonstrated a significantly lower false-positive rate (1%) than the ESC criteria (24%).

Radiofrequency Ablation in the Sinus of Valsalva for Ventricular Arrhythmia in Pediatric Patients.

The need to perform catheter ablation of ventricular arrhythmia from within the sinuses of Valsalva in a pediatric patient is uncommon. This has been reported in adults, but there are little data about the feasibility, safety or efficacy of catheter ablation in the sinuses of Valsalva in the pediatric patients. This is a retrospective review of all patients aged 18 years or less, at two separate institutions with no structural heart disease that underwent an ablation procedure for ventricular arrhythmia mapped to the sinus of Valsalva from 2010 to 2015. We identified 8 total patients meeting inclusion criteria. Median age was 16 years and the median weight was 61 kg. All patients were symptomatic or had developed arrhythmia-induced ventricular dysfunction. Ablation was performed in the left sinus in 4 patients and the right sinus in 4 patients. No ablations were required in the non-coronary sinus. All 8 patients had an acutely successful ablation using radiofrequency energy. There were no complications. At a mean follow-up of 7 months (4-15 months), all patients were known to be living. Follow-up data regarding arrhythmia were available in 6 of the 8 patients, and none had recurrence of their ventricular arrhythmia off of all antiarrhythmic medications. Radiofrequency catheter ablation of ventricular arrhythmia in the sinus of Valsalva can be done safely and effectively in pediatric patients.

Tachycardia detection using smartphone applications in pediatric patients.

OBJECTIVES: To use smartphone applications (apps) to measure heart rates during supraventricular tachycardia (SVT) in pediatric patients and compare them with heart rates measured by standard electrocardiogram (ECG). STUDY DESIGN: Patients <18 years of age (n = 26) undergoing an electrophysiology study were enrolled. During the study, heart rates were measured at baseline and during SVT by the use of 2 smartphone apps. The obtained heart rates were compared with a simultaneous standard ECG. Pearson correlation coefficient (r) was used to compare the accuracy of the apps with ECG. RESULTS: At baseline, 33 heart rates were obtained with apps and all were within ±4 beats per minute (bpm) of the ECG heart rate. During SVT, 38 heart rate measurements were attempted during 21 SVT events in 18 patients. App 1 failed to provide a measured heart rate in 11 of 21 attempts. The 10 heart rates obtained had an r of 0.56. When tachycardia rates were <210 bpm, accuracy increased (r = 0.86) and when tachycardia rates were <200 bpm, the accuracy increased further (r = 0.99). App 2 failed to provide a measured heart rate in 12 of 17 attempts. The 5 heart rates obtained had an r of -0.43. CONCLUSIONS: During tachycardia, neither of the 2 apps consistently determined an accurate heart rate at rates >200 bpm. The apps tested should not be considered an accurate tool for assessment of heart rates during SVT in pediatric patients. Select apps may have utility detecting slower SVT or confirming normal heart rates with further validation.

Wolff-Parkinson-White syndrome and adenosine response in pediatric patients.

BACKGROUND: Adenosine administration to patients with Wolff-Parkinson-White (WPW) usually increases preexcitation and therefore may be diagnostic for WPW syndrome when the electrocardiogram (ECG) is questionable. We aimed to determine the adenosine response in pediatric patients with WPW pattern on ECG and whether blocked accessory pathway (AP) conduction with adenosine correlated with nonrapid AP conduction measured by invasive electrophysiology study (EPS). METHODS: All patients with WPW ≤ 18 years of age who underwent EPS over a 5-year period were identified. The adenosine response during atrial pacing was characterized as blocked or continued AP conduction. Invasive data were obtained during atrial pacing and atrial fibrillation. Conduction through the AP to a cycle length ≤ 250 ms was considered rapid; otherwise patients were nonrapid. The sensitivity, specificity, and positive (PPV) and negative predictive value were calculated for blocked AP conduction to identify nonrapid baseline AP conduction during EPS. RESULTS: There were 59 patients included and nine (15%) had blocked AP conduction with adenosine. Five of these nine had WPW syndrome and four had fasciculoventricular APs. All nine patients had nonrapid conduction on baseline EPS. Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 100%, a PPV of 100%. CONCLUSIONS: In these pediatric patients with WPW pattern on ECG, a significant minority blocked AP conduction with adenosine and this finding had 100% specificity and PPV for nonrapid baseline antegrade AP conduction. The finding of blocked AP conduction with adenosine may aid in risk stratification.

Pediatric Epicardial Devices: Early and Midterm Outcomes.

BACKGROUND: Lead performance is suboptimal in young patients and a main cause of device system failure. Our objective was to assess early and midterm outcomes after epicardial device implantation in a contemporary pediatric cohort. METHODS: A total of 116 consecutive pediatric patients underwent 137 epicardial device implantations from 2010 to 2019. Forty pacemakers and 97 implantable cardioverter defibrillators (ICDs) were implanted. Lead failure was defined as leads repaired, replaced, or abandoned due to fracture, dislodgement, or dysfunction. Freedom from device system failure was determined using Kaplan-Meier analysis. RESULTS: Mean age at implantation was 10 ± 5 years, 46 (34%) were younger than 8 years old, 41 (30%) had prior cardiac surgery, and 38 (28%) had prior devices. Main indications were acquired heart block (17/40 [43%]), sinus node dysfunction (14/40 [35%]), and congenital heart block (7/40 [18%]) for pacemakers, and hypertrophic cardiomyopathy (46/97 [47%]), long QT syndrome (31/97 [32%]), and ventricular arrhythmia (17/97 [18%]) for ICDs. There were no early deaths. Three-year freedom from device system failure was 80% (95% CI 73%, 88%) for all patients and 88% (95% CI 79%, 99%) for patients <8 years old. Device system failure causes included lead fracture (20/34 [59%]), lead dysfunction (5/34 [15%]), lead dislodgement (5/34 [15%]), infection (3/34 [9%]), and pericarditis (1/34 [3%]). Reintervention was required in 26/34 (76%) device system failures. CONCLUSIONS: Epicardial device implantation is safe, shows acceptable midterm outcomes in children, and is an effective option in patients younger than 8 years old. Close device surveillance continues to be essential to detect lead failure early and ensure timely reintervention.

Contemporary Early Postoperative Cone Repair Outcomes for Patients With Ebstein Anomaly.

OBJECTIVE: To describe the early postoperative outcomes after cone repair (CR) for Ebstein anomaly (EA) across the age spectrum. PATIENTS AND METHODS: For this study, 284 patients from 1 to 73 years of age who underwent CR at Mayo Clinic from June 1, 2007, to December 21, 2018, were separated by age group (1-<4, 4-<19, 19-<40, and 40+ years) and by disease severity for analysis. Outcomes of interest included death, reoperation, readmission, early postoperative complications, cardiac intensive care unit and hospital length of stay, and need for superior cavopulmonary anastomosis. RESULTS: Mortality within 30 days was 0%. The reoperation rate was 4.9% (n=14) and the median hospital length of stay was 5 days, with no statistical difference between ages at time of CR or severity groups. The readmission rate was 2% (n=6). Postoperative complications were seen in 8.8% (n=25) of cases overall, with higher rates in the youngest age group (21%, P<.001). Superior cavopulmonary anastomosis was most common in the youngest age group (37% vs 17% overall, P<.001) and in those with severe disease (35%, P<.001). CONCLUSION: Children and adults with Ebstein anomaly have very good early postoperative outcomes with a less than 10% complication and reoperation rate and very low mortality following cone reconstruction. In the setting of good and stable right ventricle function and no symptoms of heart failure or cyanosis, waiting for CR until 4 years of age may minimize early postoperative complications and need for superior cavopulmonary anastomosis.

Septal Myectomy Outcomes in Children and Adolescents With Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: Little data exist regarding characteristics and outcomes of pediatric patients undergoing septal myectomy. We evaluated this in a large referral population. METHODS: Septal myectomy was performed in 199 consecutive patients aged ≤18 years with obstructive hypertrophic cardiomyopathy from January 1, 1976, to June 30, 2021. RESULTS: Median age was 13 years (interquartile range [IQR], 8-15 years). Left ventricular myectomy approaches included transaortic (163 of 198 [82%]), transapical (16 of 198 [8%]), and combined (19 of 198 [10%]). Right ventricular interventions included myectomy (13 of 199 [7%]) and patch reconstruction of the outflow tract (15 of 199 [8%]). Maximum left ventricular outflow tract gradients decreased after myectomy (prebypass: 50 mm Hg [IQR, 31-73 mm Hg] vs postbypass: 4 mm Hg [IQR, 0-9 mm Hg], P < .001), and this was sustained long-term (5 mm Hg [IQR, 5-10 mm Hg] at 10 years). Iatrogenic aortic and mitral valve injuries occurred in 13 of 199 (7%) and 1 of 199 (1%), respectively; however, all were successfully repaired. Operative mortality was 2 of 199 (1%). The cumulative incidence of redo myectomy was low, at 5.8% at 5 and 8.3% at 10 years. Redo myectomy patients had higher maximum left ventricular outflow tract gradients on echocardiography at predischarge and 1 year and were younger at the index operation (8 years [IQR, 2.5-10 years] vs 13 years [IQR, 9-16 years], P < .001). Overall survival at 10 years was 90%, relative to 47% in a previously reported pediatric nonoperative cohort. CONCLUSIONS: Pediatric septal myectomy provides safe, effective, and durable relief of ventricular outflow tract obstruction. Iatrogenic valve injury remains a low but nonnegligible risk. Recurrent obstruction requiring redo myectomy is infrequent and can be identified early. Long-term survival in this pediatric septal myectomy cohort appears to fare better than pediatric hypertrophic cardiomyopathy cohorts managed nonoperatively.

Risk stratification in Wolff-Parkinson-White syndrome: the correlation between noninvasive and invasive testing in pediatric patients.

BACKGROUND: In Wolff-Parkinson-White (WPW) syndrome, rapid antegrade conduction of atrial tachyarrhythmias can result in ventricular fibrillation and sudden death. Antegrade conduction can be assessed through noninvasive testing or invasive electrophysiology study (EPS). We aimed to determine the correlation between noninvasive testing and EPS in a pediatric WPW population. METHODS: All WPW patients <21 years who underwent EPS over a 10-year period were identified. Noninvasive testing reviewed included electrocardiogram, Holter, and exercise stress test (EST). Patients were classified as low-risk if preexcitation was lost during any test. EPS data reviewed included antegrade conduction during atrial pacing and atrial fibrillation. Conduction through the accessory pathway (AP) to a cycle length ≤ 250 ms was considered rapid, otherwise patients were nonrapid. Sensitivity, specificity, positive (PPV), and negative predictive value (NPV) of noninvasive testing to correctly identify nonrapid conduction was calculated. RESULTS: There were 135 EPS. Twenty-four patients (18%) were classified low-risk noninvasively. Two of the 24 (8%) had rapid conduction at baseline EPS. The sensitivity, specificity, PPV, and NPV of low-risk noninvasive testing to predict nonrapid conduction was 22%, 94%, 92%, and 31%, respectively. Sixteen of the 24 had low-risk EST and none had rapid conduction at baseline EPS. The specificity and PPV of low-risk EST were 100%. CONCLUSION: Loss of preexcitation during noninvasive testing had high specificity and PPV for nonrapid antegrade conduction during baseline EPS. Abrupt loss of preexcitation during EST was a highly reliable noninvasive marker of nonrapid AP conduction at baseline in our pediatric WPW patients.