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Background: Frequent premature ventricular contractions (PVCs) in children are usually considered benign. Symptoms and left ventricular dysfunction are indications for treatment with antiarrhythmic drugs., Objective: This study aimed to evaluate the efficacy of flecainide vs metoprolol in reducing PVCs in children., Methods: A randomized open-label crossover trial was conducted of children with a PVC burden of >15% on Holter monitoring successively treated with metoprolol and flecainide, or vice versa, with a drug-free interval of at least 2 weeks. Holter measurements were repeated before and after the start of the antiarrhythmic drug., Results: Sixty patients were screened; 19 patients could be included. Median age was 13.9 years (interquartile range, 5.5 years). Mean baseline PVC burden was 21.7% (n = 18; SD ± 14.0) before the start of flecainide and 21.2% (n = 17; SD ± 11.5) before the start of metoprolol. In a mixed model analysis, the estimated mean reduction in PVC burden was 10.6 percentage points (95% CI, 5.8-15.3) for flecainide and 2.4 percentage points (95% CI,2.7-7.5) for metoprolol, with a significant difference of 8.2 percentage points (95% CI, 0.86-15.46; P = .031). Exploratory analysis revealed that 9 of 18 patients treated with flecainide and 1 of 17 patients treated with metoprolol had a reduction to a PVC burden below 5%. No discriminating factors between flecainide responders and nonresponders were found; the mean plasma level was not significantly different (0.34 mg/L vs 0.52 mg/L; P = .277)., Conclusion: In children with frequent PVCs, flecainide led to a significantly greater reduction of PVC burden compared with metoprolol. Flecainide was effective in only a subgroup of patients, which appears to be unrelated to the plasma level., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Aims: To evaluate the effect of electro-anatomical mapping on success rate and fluoroscopy time in ablation of supraventricular tachycardia substrates in a large group of children., Methods: Patients referred from multiple centres in the Netherlands and who received a first ablation for supraventricular tachycardia substrates in the Leiden University Medical Center between 2014 and 2020 were included in this retrospective cohort study. They were divided in procedures in patients with fluoroscopy and procedures in patients using electro-anatomical mapping., Results: Outcomes of ablation of 373 electro-anatomical substrates were analysed. Acute success rate in the fluoro-group (n = 170) was 95.9% compared to 94.5% in the electro-anatomical mapping group (n = 181) (p = 0.539); recurrence rate was 6.1% in the fluoro-group and 6.4% in the electro-anatomical mapping group (p = 0.911) after a 12-months follow-up. Redo-ablations were performed in 12 cases in the fluoro-group and 10 cases in the electro-anatomical mapping group, with a success rate of 83.3% versus 80.0%, resulting in an overall success rate of 95.9% in the fluoro-group and 92.8% in the electro-anatomical mapping group (p = 0.216) after 12 months. Fluoroscopy time and dose area product decreased significantly from 16.00 ± 17.75 minutes (median ± interquartile range) to 2.00 ± 3.00 minutes (p = 0.000) and 210.5 µGym 2 ± 249.3 to 32.9 µGym 2 ± 78.6 (p = 0.000), respectively. In the fluoro-group, four complications occurred (2.0%) and in the electro-anatomical mapping group no complications occurred., Conclusion: These results demonstrate that ablations of supraventricular tachycardia substrates in children remain a highly effective and safe treatment after the introduction of electro-anatomical mapping as a standard of care, while significantly reducing fluoroscopy time and dose area product.

The aim of the study is to compare the efficacy of flecainide, beta-blockers, sotalol, and verapamil in children with frequent PVCs, with or without asymptomatic VT. Frequent premature ventricular complexes (PVCs) and asymptomatic ventricular tachycardia (VT) in children with structurally normal hearts require anti-arrhythmic drug (AAD) therapy depending on the severity of symptoms or ventricular dysfunction; however, data on efficacy in children are scarce. Both symptomatic and asymptomatic children (≥ 1 year and < 18 years of age) with a PVC burden of 5% or more, with or without asymptomatic runs of VT, who had consecutive Holter recordings, were included in this retrospective multi-center study. The groups of patients receiving AAD therapy were compared to an untreated control group. A medication episode was defined as a timeframe in which the highest dosage at a fixed level of a single drug was used in a patient. A total of 35 children and 46 medication episodes were included, with an overall change in PVC burden on Holter of -4.4 percentage points, compared to -4.2 in the control group of 14 patients. The mean reduction in PVC burden was only significant in patients receiving flecainide (- 13.8 percentage points; N = 10; p = 0.032), compared to the control group and other groups receiving beta-blockers (- 1.7 percentage points; N = 18), sotalol (+ 1.0 percentage points; N = 7), or verapamil (- 3.9 percentage points; N = 11). The efficacy of anti-arrhythmic drug therapy on frequent PVCs or asymptomatic VTs in children is very limited. Only flecainide appears to be effective in lowering the PVC burden.

Background: To evaluate QT-interval dynamics in patients and in drug safety analysis, beat-to-beat QT-interval measurements are increasingly used. However, interobserver differences, aberrant T-wave morphologies and changes in heart axis might hamper accurate QT-interval measurements., Objective: To develop and validate a QT-interval algorithm robust to heart axis orientation and T-wave morphology that can be applied on a beat-to-beat basis., Methods: Additionally to standard ECG leads, the root mean square (ECGRMS), standard deviation and vectorcardiogram were used. QRS-onset was defined from the ECGRMS. T-wave end was defined per individual lead and scalar ECG using an automated tangent method. A median of all T-wave ends was used as the general T-wave end per beat. Supine-standing tests of 73 patients with Long-QT syndrome (LQTS) and 54 controls were used because they have wide ranges of RR and QT-intervals as well as changes in T-wave morphology and heart axis orientation. For each subject, automatically estimated QT-intervals in three random complexes chosen from the low, middle and high RR range, were compared with manually measured QT-intervals by three observers., Results: After visual inspection of the randomly selected complexes, 21 complexes were excluded because of evident noise, too flat T-waves or premature ventricular beats. Bland-Altman analyses of automatically and manually determined QT-intervals showed a bias of <4ms and limits of agreement of ±25ms. Intra-class coefficient indicated excellent agreement (>0.9) between the algorithm and all observers individually as well as between the algorithm and the mean QT-interval of the observers., Conclusion: Our automated algorithm provides reliable beat-to-beat QT-interval assessment, robust to heart axis and T-wave morphology.

Aims: To assess the risk factors for left ventricular (LV) dysfunction in a paediatric population with idiopathic frequent premature ventricular contractions (PVCs) and asymptomatic ventricular tachycardias (VTs)., Methods and Results: Paediatric patients with the diagnosis of idiopathic frequent PVCs and asymptomatic VTs were retrospectively evaluated. Frequent PVCs were defined as ≥5% on 24 h Holter recording. Left ventricular dysfunction was defined as a shortening fraction of ≤28%. Seventy-two children were identified. Six patients showed LV dysfunction at diagnosis [age 10 ± 7 years, 2 (33%) had symptoms such as syncope, palpitations, fatigue, and dizziness], and 66 showed normal LV function [age 8 ± 6 years, 22 (33%) with symptoms]. Patients with LV dysfunction had a higher percentage of PVCs on Holter recordings (47 ± 16 vs. 16 ± 11%, P = 0.006), higher prevalence of VT [5 (83%) vs. 27 (41%), P = 0.045] and sustained ventricular tachycardia (sVT) [3 (50%) vs. 4 (6%), P = 0.001], and a higher number of couplets [6 (100%) vs. 34 (52%), P = 0.030]. In patients with LV dysfunction, two responded to medication (Classes Ic and II) and five underwent ablation, of which one was unsuccessful. During follow-up, LV function normalized in five of six patients. In patients with a normal function, none developed LV dysfunction during the follow-up., Conclusion: In children with idiopathic PVCs and asymptomatic VTs, development of LV dysfunction is associated with a higher burden of PVCs, the presence of sVTs, and couplets. Left ventricular dysfunction appears to be reversible if the burden of PVCs is decreased by medication or ablation., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

A newborn had a tight, dry skin with multiple fissures, an eclabium and ectropion. Breathing was compromised and regulation of body temperature was impaired. We described the patient as a 'collodion baby'. After genetic investigation the diagnosis congenital ichtyosis, recessive type 1 was made. At 12 months of age, the skin symptoms were significantly diminished.

Aims: Tissue Doppler imaging (TDI) enables assessment of velocities and timings within the left (LV) and the right (RV) ventricle with high temporal resolution. Knowledge on normal age-related values of peak systolic velocities and timings in healthy children may optimize the benefit of device-based therapies in paediatric patients with heart failure., Methods and Results: A total of 123 healthy children (from 1 month to 18 years old) underwent TDI evaluation of the RV and LV. Peak systolic velocity and time to peak systolic velocity were assessed at the basal LV lateral wall, inter-ventricular septum (IVS), RV free wall (RVFW), and at the RV outflow tract (RVOT). Intra-ventricular time differences were calculated. Regression analysis was performed to assess the age dependency of the ventricular mechanics. Median peak velocities were: LV lateral wall: 6.3 cm/s (inter-quartile range (IQR): 5.1-7.9 cm/s); IVS: 6.0 cm/s (5.4-6.7 cm/s); RVFW: 10.2 cm/s (8.9-11.3 cm/s); RVOT: 7.2 cm/s (6.0-8.2 cm/s). Timings of peak systolic velocities were: LV lateral wall: 101 ms (91-112 ms); IVS: 114 ms (100-128 ms); RVFW: 177 ms (157-194 ms); RVOT: 100 ms (88-113 ms). Timings and peak velocities significantly increased with age at both ventricles. No relevant time difference was observed within the LV, whereas a considerable time delay was observed within the RV between the RVFW and the IVS (62 ms, IQR: 45-74 ms) and between the RVFW and the RVOT (74 ms, IQR: 59-93 ms)., Conclusion: The present evaluation provides TDI-derived physiological values on normal LV and RV mechanics of healthy children. Within the LV, no relevant time difference was observed, whereas a considerable mechanical delay is observed within the healthy RV.

The epinephrine test has been shown to be a powerful tool to predict the genotype of congenital long QT syndrome (LQTS). The aim of this study was to evaluate its role in the diagnosis and management of LQTS in children. The test (using the Shimizu protocol) was conducted in patients with some evidence of LQTS but in whom clinical and management decisions were challenging (n = 41, age 9.6 +/- 3.9 years, 19 female). LQT1, LQT2, and negative responses to epinephrine were obtained in 16, 5, and 20 subjects, respectively. LQTS gene positivity was obtained in two subjects. Beta-blocker therapy was started in all subjects with a positive epinephrine response (n = 21) and in some negative responders because of their strong LQTS phenotype (n = 10). No therapy was given to the subset with less convincing features of LQTS who had also responded negatively to epinephrine (n = 10). Follow-up for 3.0 +/- 2 years was uneventful in both management groups. Due to the discordance with genotyping, the epinephrine test cannot be used to diagnose genotype-positive LQTS but when used in combination with phenotype assessment and genetic screening, it could enable better management decisions.

Two infants were referred to the emergency ward: a 3.5-month-old male infant in whom sweating and shortness of breath were seen during drinking and crying, and a 5-week-old girl that had drunk less the past day, moaned while breathing for the past few hours and had become increasingly drowsy. They were not suffering from an infection but from a dilated cardiomyopathy based on an anomalous origin of the left coronary artery from the pulmonary artery. The left coronary artery was then implanted in the aorta surgically. Both patients recovered well. Airway tract infection and sepsis are obvious common differential diagnostic considerations when confronted with a tachy-dyspnoeic infant. However, a cardiac cause, such as a dilated cardiomyopathy with cardiac decompensation, must also be considered as the presentation can be similar. The abnormal origin of the left coronary artery from the pulmonary artery is one of the few readily well treatable causes.

We describe the importance of angiographic identification of collateral venous channels by balloon occlusion venography after bidirectional cavopulmonary connections. Use of the balloon occlusion technique is essential for identification of these vessels, as they can easily be missed by standard venous angiography, with possible clinical consequences for postoperative management.

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

The use of catheterization for the diagnosis of congenital heart disease has decreased dramatically in the last decade. The development of two-dimensional Doppler echocardiography has changed the practice of pediatric cardiology, combining exquisite imaging with Doppler-derived hemodynamic information. Angiographic cardiac imaging is required for those areas inaccessible to ultrasonography, such as the distal great arteries and abnormal venous connections, and also in complex postoperative conditions. To further decrease the need for angiography, transesophageal echocardiography, magnetic resonance imaging, radionuclide angiography, and spiral computed tomography have been employed as noninvasive substitutes in those areas. Of these modalities, magnetic resonance imaging appears to be the most promising. Currently, angiography is used almost exclusively for interventional purposes. The increased imaging versatility afforded by these new modalities has important financial consequences, and requires a responsible economic approach. This article compares the role and requirements of invasive cardiac imaging in the catheterization laboratory with those of these newly developed techniques.