The influence of exercise and postural changes on ventricular repolarization in the long QT syndrome: a systematic scoping review.

Current exercise recommendations make it difficult for long QT syndrome (LQTS) patients to adopt a physically active and/or athletic lifestyle. The purpose of this review is to summarize the current evidence, identify knowledge gaps, and discuss research perspectives in the field of exercise and LQTS. The first aim is to document the influence of exercise training, exercise stress, and postural change interventions on ventricular repolarization in LQTS patients, while the second aim is to describe electrophysiological measurements used to study the above. Studies examining the effects of exercise on congenital or acquired LQTS in human subjects of all ages were included. Systematic searches were performed on 1 October 2021, through PubMed (NLM), Ovid Medline, Ovid All EBM Reviews, Ovid Embase, and ISI Web of Science, and limited to articles written in English or French. A total of 1986 LQTS patients and 2560 controls were included in the 49 studies. Studies were mainly case-control studies (n = 41) and examined exercise stress and/or postural change interventions (n = 48). One study used a 3-month exercise training program. Results suggest that LQTS patients have subtype-specific repolarization responses to sympathetic stress. Measurement methods and quality were found to be very heterogeneous, which makes inter-study comparisons difficult. In the absence of randomized controlled trials, the current recommendations may have long-term risks for LQTS patients who are discouraged from performing physical activity, rendering its associated health benefits out of range. Future research should focus on discovering the most appropriate levels of exercise training that promote ventricular repolarization normalization in LQTS.

Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study.

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns. METHODS: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands. RESULTS: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia. CONCLUSIONS: ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Ventricular Arrhythmias and Sudden Death Following Percutaneous Pulmonary Valve Implantation in Pediatric Patients.

Reports have suggested a transient increase in ventricular ectopy early after percutaneous pulmonary valve implantation (PPVI). Little is known about the potential for more serious ventricular arrhythmias (VA) in children who undergo PPVI. We sought to evaluate the incidence of severe VA following PPVI in a pediatric population and to explore potential predictive factors. A retrospective cohort study was conducted of patients who underwent PPVI under 20 years of age in our institution from January 2007 to December 2019. The primary outcome of severe VA was defined as sustained and/or hemodynamically unstable ventricular tachycardia (VT), inducible sustained VT, or sudden death of presumed arrhythmic etiology. A total of 21 patients (mean age 16.2 ± 2.1 years; 66.7% male) underwent PPVI. The majority of patients (N = 15; 71.4%) had tetralogy of Fallot (TOF) or TOF-like physiology, with the most common indication being pulmonary insufficiency (N = 10; 47.6%). During a median follow-up of 29.6 months (IQR 10.9-44.0), severe VA occurred in 3 (14.3%) patients aged 15.6 (IQR 14.7-16.1) a median of 12.3 months (IQR 11.2-22.3) after PPVI. All events occurred in patients with TOF-like physiology following Melody valve implant. In conclusion, severe VA can occur long after PPVI in a pediatric population, particularly in those with TOF-like physiology. Further studies are required to elucidate underlying mechanisms and assess strategies to mitigate risks.

Diagnostic Efficacy of a Single-Lead Ambulatory 14-Day ECG Monitor in Symptomatic Children.

BACKGROUND: The CardioSTAT is a single-lead ambulatory electrocardiography monitor that has been validated for use in adult patients. Recording is made through 2 electrodes positioned in a lead-I configuration, and the device allows monitoring for 2, 7, or 14 days. We sought to investigate the efficacy of this device in children with paroxysmal palpitations. METHODS: In phase I, the quality of tracings from simultaneous CardioSTAT recordings and D1-lead recordings of a standard 12-lead electrocardiography machine in 23 children were compared. Phase II was a prospective observational cohort study comparing arrhythmia detection using the CardioSTAT vs currently used devices (24-hour Holter monitor and the Cardiomemo loop recorder) in 52 children complaining of palpitations. RESULTS: In Phase I, all but 3 rhythm strips were correctly identified. The pacing spikes on 3 strips were not adequately identified by the observers for the CardioSTAT recording. In Phase II, symptomatic episodes were reported in 42%, 73%, and 100% of subjects during monitoring with the Holter, Cardiomemo, and CardioSTATdevices, respectively. An abnormal rhythm was detected in 13%, 23%, and 35% of subjects by the Holter, Cardiomemo, and CardioSTAT monitors, respectively. The underlying rhythm during symptomatic events was determined in 90% of cases with the CardioSTAT monitor, whereas it was determined in only 19% and 29% of cases using the Holter and Cardiomemo monitors, respectively. CONCLUSIONS: The CardioSTAT monitor provided good-quality tracings and was superior to the 24-hour Holter monitor and the Cardiomemo loop recorder in determining the presence or absence of pathologic arrhythmia in the study cohort.

CONTEXTE: Le CardioSTAT est un moniteur d'électrocardiographie ambulatoire à dérivation unique dont l'utilisation a été validée chez les patients adultes. L'enregistrement se fait au moyen de 2 électrodes positionnées dans une configuration de type « dérivation I ¼, et l'appareil permet un suivi pendant 2, 7 ou 14 jours. Nous avons cherché à étudier l'efficacité de cet appareil chez les enfants atteints de palpitations paroxystiques. MÉTHODOLOGIE: Dans la phase I, la qualité des tracés provenant d'enregistrements simultanés de CardioSTAT et d'enregistrements de la dérivation I d'un appareil d'électrocardiographie standard à 12 dérivations a été comparée chez 23 enfants. La phase II était une étude de cohorte observationnelle prospective comparant la détection de l'arythmie à l'aide du moniteur CardioSTAT par rapport aux appareils utilisés actuellement (moniteur Holter à surveillance sur 24 heures et enregistreur d'événements portatif Cardiomémo) chez 52 enfants se plaignant de palpitations. RÉSULTATS: Dans la phase I, toutes les bandes d'enregistrement sauf trois ont été correctement identifiées. Les spicules de stimulation n'ont pas été correctement détectés par les observateurs sur trois bandes d'enregistrement du moniteur CardioSTAT. Dans la phase II, des épisodes symptomatiques ont été signalés chez 42 %, 73 % et 100 % des sujets pendant la surveillance avec les appareils Holter, Cardiomémo et CardioSTAT, respectivement. Un rythme anormal a été détecté chez 13 %, 23 % et 35 % des sujets par les moniteurs Holter, Cardiomémo et CardioSTAT, respectivement. Le rythme sous-jacent pendant les événements symptomatiques a été déterminé dans 90 % des cas avec le moniteur CardioSTAT, alors qu'il n'a été déterminé que dans 19 % et 29 % des cas, respectivement, avec les moniteurs Holter et Cardiomémo. CONCLUSIONS: Le moniteur CardioSTAT a fourni des tracés de bonne qualité et s'est révélé supérieur à l'appareil Holter à surveillance sur 24 heures et à l'enregistreur d'événements portatif Cardiomémo pour déterminer la présence ou l'absence d'arythmie pathologique dans la cohorte étudiée.

New developments in catheter ablation for patients with congenital heart disease.

Introduction: There are numerous challenges to catheter ablation in patients with congenital heart disease (CHD), including access to cardiac chambers, distorted anatomies, displaced conduction systems, multiple and/or complex arrhythmia substrates, and excessively thickened walls, or interposed material. Areas covered: Herein, we review recent developments in catheter ablation strategies for patients with CHD that are helpful in addressing these challenges. Expert opinion: Remote magnetic navigation overcomes many challenges associated with vascular obstructions, chamber access, and catheter contact. Patients with CHD may benefit from a range of ablation catheter technologies, including irrigated-tip and contact-force radiofrequency ablation and focal and balloon cryoablation. High-density mapping, along with advances in multipolar catheters and interpolation algorithms, is contributing to new mechanistic insights into complex arrhythmias. Ripple mapping allows the activation wave front to be tracked visually without prior assignment of local activation times or window of interest, and without interpolations of unmapped regions. There is growing interest in measuring conduction velocities to identify arrhythmogenic substrates. Noninvasive mapping with a multielectrode-embedded vest allows prolonged bedside monitoring, which is of particular interest in those with non-sustained or multiple arrhythmias. Further studies are required to assess the role of radiofrequency needle catheters and stereotactic radiotherapy in patients with CHD.

Electrocardiographic features at rest and during exercise in young adults born preterm below 30 weeks of gestation.

BACKGROUND: Preterm birth has adverse consequences on the cardiovascular system. Whether premature birth is associated with conduction and repolarisation abnormalities past childhood and into adulthood still needs to be demonstrated. METHODS: We analyzed the ECG of young adults (23.9 ± 3.1 years) born term (≥37 weeks, n = 53) and preterm (<30 weeks, n = 49) at rest, peak exercise and 3 min into recovery during an exercise test on a cycle ergometer. We measured PR, QRS and QT intervals, calculated the corrected QT (QTc), and determined blood calcium, magnesium, potassium and fasting glucose. RESULTS: Mean gestational age was 39.7 ± 1.1 and 27.3 ± 1.3 weeks for the term and the preterm groups, respectively. Apart from an increased heart rate at rest in individuals born preterm, no significant difference was found between both groups for any other ECG parameters at rest. None of the participants had a severely prolonged QTc (>500 ms) at rest; exercise revealed severely prolonged QTc in two participants including one in the preterm group. The use of QT-prolonging medications did not influence ECG parameters in either groups. CONCLUSIONS: We observed no significant difference in electrocardiographic measurements between young adults born preterm and term. Current results do not support avoidance of QT-prolonging medications in individuals born preterm. IMPACT: Preterm birth is associated with adverse cardiovascular consequences in early adulthood, but controversial evidence exists regarding differences in electrocardiographic features between young individuals born term and preterm.This study aims to assess the differences in electrocardiographic features between young adults born term and preterm, at rest and during exercise training.In contrast with previously published data, we observed no significant difference in electrocardiographic measurements between young adults born preterm and term.Our study does not support that preterm birth itself exposes young adults to a higher risk of QT prolongation.Current results do not support avoidance of QT-prolonging medications in individuals born preterm.

Left Superior Vena Cava in the Fetus: A Rarely Isolated Anomaly.

The frequency of chromosomal anomalies among fetuses with isolated persistent left superior vena cava (PLSVC) is still debated. The objective of the present study was to assess the prevalence of genetic and morphological anomalies identified in fetuses with PLSVC. We conducted a single-center retrospective study including all fetuses diagnosed with a PLSVC between 2010 and 2017. PLSVC was categorized as isolated or associated according to antenatal diagnosis of associated congenital heart defects, hypoplastic aortic isthmus, abnormal venous/arterial connections, and extracardiac anomalies. Among 229 fetuses diagnosed with PLSVC, 39 cases (17%) were strictly isolated and no syndromic/genetic anomaly or aortic coarctation was diagnosed. Seventy-two fetuses had a cardiovascular defect with a rate of genetic anomalies of 22%, 29 had an extracardiac malformation, and 89 had both an extracardiac and a cardiovascular defect. Among fetuses with abnormal development of the arterial/venous system as the only associated anomaly such as aberrant right subclavian artery or absent ductus venosus, 22% had a genetic anomaly. Overall, sixty-five fetuses or infants had a genetic concern, including 23 aneuploidies, 15 pathogenic micro-deletions/duplications, and 5 variants of unknown significance; 12 patients had VACTERL association, and 12 heterotaxy syndrome. Seven infants had an aortic coarctation diagnosed at birth.In conclusion, a thorough prenatal ultrasound examination is paramount, and the identification of variants of the venous/arterial system in addition to PLSVC should raise suspicion for genetic or morphologic abnormalities. Invasive prenatal diagnosis with array-CGH should be offered when PLSVC is non-isolated, after a detailed ultrasound evaluation in a tertiary center.

Feasibility and safety of cryoballoon ablation for atrial fibrillation in patients with congenital heart disease.

BACKGROUND: The prevalence of atrial fibrillation (AF) is on the rise in the aging population with congenital heart disease (CHD). A few case series have described the feasibility and early outcomes associated with radiofrequency catheter ablation of AF centered on electrically isolating pulmonary veins (PV) in patients with CHD. In contrast, cryoballoon ablation has not previously been studied in this patient population despite its theoretical advantages, which include a favorable safety profile and shorter procedural time. AIM: To assess the safety and feasibility of cryoballoon ablation for AF in an initial cohort of patients with CHD. METHODS: The study population consisted of consecutive patients with CHD and cryoballoon ablation for AF at the Montreal Heart Institute between December 2012 and June 2017. Procedural complications, acute success, and 1-year freedom from recurrent AF after a single procedure with or without antiarrhythmic drugs were assessed. Procedures were performed under conscious sedation. Left atrial access was obtained via a single transseptal puncture or through an existing atrial septal defect (ASD). Cryoballoon occlusion was assessed by distal injection of 50% diluted contrast into the pulmonary vein. At least one 240-second cryothermal application was performed upon obtaining complete pulmonary vein occlusion. Following ablation, patients were routinely followed at outpatient visits at 1, 3, 6, and 12 mo, and then annually. RESULTS: Ten patients, median age 57.9 (interquartile range 48.2-61.7) years, 60% female, met inclusion criteria and were followed for 2.8 (interquartile range 1.4-4.5) years. Two had moderately complex CHD (sinus venosus ASD with partial anomalous pulmonary venous return; aortic coarctation with a persistent left superior vena cava), with the remainder having simple defects. AF was paroxysmal in 8 (80.0%) and persistent in 2 (20.0%) patients. The pulmonary vein anatomy was normal in 6 (60.0%) patients. Four had left common PV (n = 3) and/or 3 right PV (n = 2). Electrical pulmonary vein isolation (PVI) was acutely successful in all. One patient had transient phrenic nerve palsy that recovered during the intervention. No major complication occurred. One year after a single ablation procedure, 6 (60%) patients remained free from AF. One patient with recurrent AF had recovered pulmonary vein conduction and underwent a second PVI procedure. A second patient had ablation of an extra-pulmonary vein trigger for AF. CONCLUSION: Cryoballoon ablation for AF is feasible and safe in patients with simple and moderate forms of CHD, with an excellent acute success rate and modest 1-year freedom from recurrent AF.

Atrial fibrillation in adults with congenital heart disease.

A convergence of epidemiological and clinical features has prompted a precipitous rise in the prevalence of atrial fibrillation (AF) in the expanding population of adults with congenital heart disease. Herein, we synthesize the current state of knowledge on epidemiological features, associated morbidities, and pathophysiological insights regarding AF in adults with congenital heart disease. Management issues are examined including surgical, pharmacological, and catheter-based therapies. Throughout, knowledge gaps and avenues for future research are identified. Although AF has been coined the next arrhythmic epidemic to strike adults with congenital heart disease, it has already usurped atrial macroreentrant tachycardia as the most common presenting arrhythmia over the age of 50 years. Much remains to be discovered about the mitigating role of types of congenital defects, residual hemodynamic lesions, surgical sequelae, and ramifications of shunts and cyanosis on mechanisms and determinants of AF. Thromboprophylaxis is the cornerstone of pharmacological management, with anticoagulation recommended in patients with moderate or complex congenital heart disease and those with significant valve disease or risk factors for stroke. Considering the limited success with antiarrhythmic drugs, catheter ablation is increasingly performed. Non-pulmonary vein sources, focal and reentrant arrhythmias, appear to be important triggers for AF in this population. As such, they should be identified and addressed during catheter ablation interventions. The nascent literature on electrical isolation of pulmonary veins suggests that it is feasible and safe, although initial success rates appear to be modest. A more thorough understanding of underlying mechanisms and substrates carries the potential to further improve outcomes.

Cryoablation for Perinodal Arrhythmia Substrates in Patients With Congenital Heart Disease and Displaced Atrioventricular Conduction Systems.

OBJECTIVES: The purpose of this study was to assess the safety and efficacy of cryoablation for perinodal substrates in patients with congenital heart disease (CHD) and a displaced atrioventricular (AV) conduction system or an AV conduction system location that was difficult to predict. BACKGROUND: Catheter ablation for perinodal arrhythmias in patients with CHD may incur higher risks due to unconventional or difficult to predict locations of the AV conduction system. Cryoablation carries theoretical advantages for such patients but has not been studied in this setting. METHODS: A total of 35 patients with CHD underwent cryoablation for perinodal substrates at the Montreal Heart Institute between 2006 and 2016. Ten of these patients, age 33 ± 13 years, 60% male, had AV conduction systems that were displaced or of uncertain location and underwent cryoablation (6-mm electrode-tip catheter) for 12 perinodal arrhythmias: AV nodal re-entrant tachycardia (n = 4), non-automatic focal atrial tachycardia (n = 4), septal intra-atrial re-entrant tachycardia (n = 3), and para-Hisian automatic focal atrial tachycardia (n = 1). Four patients had single-ventricle physiology and had undergone Fontan palliation (3 atriopulmonary and 1 intracardiac total cavopulmonary connection), 4 underwent repair of AV septal defects, 1 had congenitally corrected transposition of the great arteries (TGA), and 1 had TGA with a Mustard baffle. RESULTS: Cryoablation was acutely successful in 9 of 12 targeted arrhythmias (75%) with no procedural complication. Crossover to radiofrequency ablation successfully eliminated the remaining 3 arrhythmias at sites deemed safe by cryoablation, with no complication. Over a follow-up period of 26 (interquartile range: 15 to 64) months, 1 of 9 successfully cryoablated arrhythmias recurred. CONCLUSIONS: Cryoablation is feasible, safe, and moderately effective for perinodal arrhythmia substrates in patients with various forms of CHD associated with AV conduction systems that are displaced or in locations that cannot be reliably predicted.

Atrial fibrillation in young patients.

INTRODUCTION: Atrial fibrillation (AF) is the most frequent arrhythmia worldwide. While mostly seen in elderly, it can also affect young adults (≤ 45 years of age), older adolescent, and children. Areas covered: The aim of this review is to provide an overview of the current management of AF in young patients. Specific issues arise over diagnostic workup as well as antiarrhythmic and anticoagulation therapies. The future management and diagnostic strategies are also discussed. Expert commentary: Management of AF in the young adult is largely extrapolated from adult studies and guidelines. In this population, AF could reveal a genetic pathology (e.g. Brugada, Long QT or Short QT syndromes) or be the initial presentation of a cardiomyopathy. Therefore, thorough workup in the young population to eliminate potential malignant pathology.

Dynamic QT Interval Changes from Supine to Standing in Healthy Children.

BACKGROUND: QT-interval variations in response to exercise-induced increases in heart rate have been reported in children and adults in the diagnosis of long QT syndrome (LQTS). A quick standing challenge has been proposed as an alternative provocative test in adults, with no pediatric data yet available. METHODS: A standing test was performed in 100 healthy children (mean age, 9.7 ± 3.1 years) after 10 minutes in a supine position with continuous electrocardiographic recording. QT intervals were measured at baseline, at maximal heart rate, at maximal QT, and at each minute of a 5-minute recovery while standing. Measurements were taken in leads II/V5 and were corrected for heart rate (QTc). RESULTS: On standing, the heart rate increased by 29 ± 10 beats per minute (bpm). The QT interval was similar at baseline and on standing (394 ± 34 ms vs 394 ± 34 ms; P = 1.0). However, QTc increased from 426 ± 21 to 509 ± 41 ms (P < 0.001). The 95th percentile for QTc at baseline and maximal heart rate was 457 ms and 563 ms, respectively. At 1 minute of recovery, the QT interval was shorter (375 ± 31 ms) compared with baseline (394 ± 34 ms; P < 0.001) and standing (394 ± 34 ms; P < 0.001). QTc reached baseline values after 1 minute of recovery and remained stable thereafter (423 ± 23 ms at 1 minute; 426 ± 22 ms at 5 minutes; P = 1.0). CONCLUSIONS: This first characterization of QTc changes on standing in children shows substantial alterations, which are greater than those seen in adults. Two-thirds of the children would have been misclassified as having LQTS by adult criteria, indicating the need to create child-specific standards.

Percutaneous Patent Ductus Arteriosus (PDA) Closure During Infancy: A Meta-analysis.

CONTEXT: Patent ductus arteriosus (PDA) is a precursor to morbidity and mortality. Percutaneous (catheter-based) closure is the procedure of choice for adults and older children with a PDA, but use during infancy (<1 year) is not well characterized. OBJECTIVE: Investigate the technical success and safety of percutaneous PDA closure during infancy. DATA SOURCES: Scopus, Web of Science, Embase, PubMed, and Ovid (Medline) were searched through December 2015 with no language restrictions. STUDY SELECTION: Publications needed to clearly define the intervention as percutaneous PDA closure during infancy (<1 year of age at intervention) and must have reported adverse events (AEs). DATA EXTRACTION: The study was performed according to the Systematic Reviews and Meta-Analysis checklist and registered prospectively. The quality of the selected studies was critically examined. Data extraction and assignment of AE attributability and severity were independently performed by multiple observers. Outcomes were agreed on a priori. Data were pooled by using a random-effects model. RESULTS: Thirty-eight studies were included; no randomized controlled trials were found. Technical success of percutaneous PDA closure was 92.2% (95% confidence interval [CI] 88.8-95.0). Overall AE and clinically significant AE incidence was 23.3% (95% CI 16.5-30.8) and 10.1% (95% CI 7.8-12.5), respectively. Significant heterogeneity and publication bias were observed. LIMITATIONS: Limitations include lack of comparative studies, lack of standardized AE reporting strategy, and significant heterogeneity in reporting. CONCLUSIONS: Percutaneous PDA closure during infancy is feasible and associated with few catastrophic AEs; however, the limitations constrain the interpretability and generalizability of the current findings.

Characteristics of premature ventricular contractions in healthy children and their impact on left ventricular function.

BACKGROUND: There are few data regarding the characteristics of premature ventricular contractions (PVCs) in healthy children and their impact on left ventricular (LV) function. OBJECTIVE: The purpose of this study was to assess the prevalence of LV systolic dysfunction in children with frequent PVCs (≥10%) and determine whether it is associated with PVC characteristics (e.g., proportion, coupling interval, width, and/or morphology). METHODS: We conducted a single-center cohort study of children with structurally normal hearts and PVC burden ≥10% by 24-hour Holter monitoring performed between 2008 and 2012. Clinical, arrhythmic, and echocardiographic data were reviewed at baseline and during follow-up. RESULTS: A total of 47 children (22 female [47%], mean age 8.2 ± 6.5 years) had a mean PVC burden of 20.9 ± 11.9% at baseline. The PVC coupling interval averaged 430 ± 110 ms, with a PVC width of 118 ± 27 ms. PVCs were monomorphic in 44 patients (94%). Although no patient had severe cardiomyopathy, 7 (15%) had reduced shortening fraction (Z-score <-2). A strong association was observed between PVC coupling interval and LV shortening fraction Z-score <-2.0 (area under the curve 0.95 ± 0.03, P <.001). A cutoff value <365 ms yielded the greatest discriminatory ability (Youden J-statistic 0.72, sensitivity 85.7%, specificity 86.5%). PVC proportion, width, and morphology were not significantly associated with LV shortening fraction. During 4.0 ± 2.8 years of follow-up, the PVC burden decreased from a median of 18% to 1.5% (P<.001). CONCLUSION: PVCs in children with structurally normal hearts are associated with a relatively benign course, with spontaneous resolution in most children. Mild LV systolic dysfunction, observed in 15%, is strongly correlated with a shorter coupling interval (<365 ms).

Left Atrial Inexcitability in Children With Congenital Lupus-Induced Complete Atrioventricular Block.

BACKGROUND: Congenital atrioventricular block is a well-established immunologic complication of maternal systemic lupus erythematosus. We sought to further characterize the electrophysiological manifestations of maternal systemic lupus erythematosus on neonatal atria. METHODS AND RESULTS: Cases of isolated congenital atrioventricular block treated at our center over the past 41 years were identified. Data were extracted from clinical charts, pacemaker interrogations, ECGs, echocardiograms, and histopathological reports, when available. Of 31 patients with isolated congenital atrioventricular block, 18 were negative for maternal antibodies and had normal epicardial atrial sensing and pacing thresholds. In contrast, 12 of 13 patients with positive maternal antibodies had epicardial pacemakers, 5 (42%) of whom had left atrial (LA) inexcitability and/or atrial conduction delay. In 3 patients, the LA could not be captured despite high-output pacing. The fourth patient had acutely successful LA appendage and left ventricular lead placement. At early follow-up, an increased delay between the surface P-wave and intracardiac atrial depolarization was observed, indicative of atrial conduction delay. The fifth patient exhibited LA lead dysfunction, with atrial under-sensing and an increased capture threshold, 2 weeks after implantation. Biopsies of LA appendages performed in 2 patients showed no evidence of atrial fibrosis or loss of atrial myocytes. CONCLUSIONS: Herein, we report previously undescribed yet prevalent electrophysiological ramifications of maternal systemic lupus erythematosus, which extend beyond congenital atrioventricular block to encompass alterations in LA conduction, including LA inexcitability. These manifestations can complicate epicardial pacemaker implantation in newborns. In the absence of histological evidence of extensive atrial fibrosis, immune-mediated functional impairment of electrical activity is suspected.

Ablation of an atriofascicular accessory pathway with a zero-fluoroscopy procedure.

A 16-year-old patient with recurrent palpitations and documented left bundle branch block superior axis wide complex tachycardia underwent an electrophysiological study and ablation with a zero-fluoroscopy procedure. The electrophysiological study showed a decremental antegrade conducting atriofascicular pathway. Three-dimensional CARTO-guided mapping of the tricuspid annulus in sinus rhythm was performed, and a distinct signal corresponding to the accessory pathway potential of the atriofascicular pathway was found in the posterolateral region. By using an SR0 sheath and a 4-mm-tip catheter, radiofrequency application was delivered at this point on the annulus and successfully eliminated conduction through the accessory pathway.

Timing of Dynamic NT-proBNP and hs-cTnT Response to Exercise Challenge in Asymptomatic Children with Moderate Aortic Valve Regurgitation or Moderate Aortic Valve Stenosis.

Patients with congenital aortic valve stenosis (AVS) can remain asymptomatic but may develop progressive and often underestimated exercise intolerance. The risk of increased left ventricular (LV) wall stress, irreversible myocardial fibrosis and sudden death in untreated patients warrants earlier intervention. The timing for curative therapy for severe AVS is clear, but optimal timing for moderate stenosis (modAS) is unknown. AVS often coexists with aortic regurgitation, which adds a volume overload to an already pressure-overloaded LV, adding an additional challenge to the estimation of disease severity. We investigated the possible value of N-terminal pro-brain natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (hs-cTnT) upon treadmill exercise challenge in children with asymptomatic modAS versus moderate regurgitation (modAR). The aim was to determine optimal timing of peak biochemical response. Blood samples were obtained at rest, and then at 20, 40 and 60 min after peak exercise comparing modAS and modAR to healthy controls. Exercise performance was equivalent in all groups, with no difference for biomarker levels at rest. The increase in NT-proBNP was significant in modAR at 40 min (99.2 ± 48.6 ng/L; p = 0.04) and 60 min into recovery (100.0 ± 53.7 ng/L; p = 0.01), but not in modAS. The increase in hs-cTnT was significant only at 60 min into recovery for modAS and modAR. NT-proBNP and hs-cTnT following exercise challenge are possible discriminant biomarkers of modAR from modAS and controls at 60 min into recovery despite comparable exercise performance. This offers a promising avenue for future stratification of aortic valve disease and optimal timing of intervention.

Risk stratification for sudden death in arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is an uncommon but increasingly recognized inherited cardiomyopathy that is associated with malignant ventricular arrhythmias and sudden cardiac death, particularly in young individuals. The implantable cardioverter-defibrillator (ICD) is widely regarded as the only treatment modality with evidence to support improved survival in patients with ARVC and secondary prevention indications. In contrast, there is no universally accepted risk stratification scheme to guide ICD therapy for primary prevention against sudden cardiac death. Potential benefits must be weighed against the considerable risks of complications and inappropriate shocks in this young patient population. This article tackles the challenges of risk stratification for sudden cardiac death in ARVC and critically appraises available evidence for various proposed risk factors. The authors' over-arching objective is to provide the clinician with evidence-based guidance to inform decisions regarding the selection of appropriate candidates with ARVC for ICD therapy.

Pharmacotherapy for inherited arrhythmia syndromes: mechanistic basis, clinical trial evidence and practical application.

In the absence of structural heart disease, sudden cardiac death is frequently caused by inherited arrhythmia syndromes, such as long QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. Managing these conditions often requires a combination of lifestyle modification, pharmacotherapy and less frequently, invasive therapy. Over the past decade, patient management has been greatly enhanced by tailored pharmacotherapy as a result of a deeper appreciation for arrhythmia mechanisms and supportive evidence from multicenter cohort studies. This article reviews current knowledge regarding drug therapy for inherited arrhythmias. Anti-arrhythmic mechanisms and available clinical evidence are highlighted while maintaining a practical perspective on patient management.

Effect of dual-chamber pacemaker implantation on aortic dilatation in patients with congenital heart block.

Ascending aortic dilatation (AoD) in patients with structurally normal hearts and congenital heart block (CHB) has been previously described. The cause and management of AoD are yet to be determined. The aim of this study was to test the hypothesis that AoD in children with CHB regresses after the implantation of cardiac dual-chamber pacemakers (PMs). The secondary hypothesis was an association between the presence of maternal antibodies (SS-A or SS-B) and the degree of aortic dilatation. Clinical data with echocardiographic correlates of patients with CHB followed at a single institution were retrospectively reviewed. Comparison of the target structures diameter was based on 3 different z-score equations, with AoD defined as a z score >2. Inclusion criteria were CHB diagnosis by 12-lead electrocardiography or Holter recording and benefit from a permanent dual-chamber PM. Excluded were patients with incomplete echocardiographic measurements, those with major structural heart defects, and those with syndromes or diseases known to be associated with AoD. There were 17 patients, diagnosed at a median age of 6 months (interquartile range 0 to 47.8). Maternal antibodies were positive in 6 patients and negative in 11. All patients underwent PM implantation at a median age of 4.5 years (interquartile range 1.4 to 7.9). AoD (z score >2.0 according to 3 different equations) was present in 35% to 59% of patients. There was a significant reduction of mean ascending aortic z score in patients with AoD from 4.66 to 3.67 (p = 0.06), from 4.82 to 2.95 (p = 0.002), and from 6.07 to 3.39 (p = 0.006) according to the various z-score equations. Most patients with positive serology had AoD, without reaching statistical significance. In conclusion, AoD is associated with CHB, more likely in infants exposed to maternal antibodies. AoD decreases after the implantation of a PM. This is probably related to the regularization of stroke volume.