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Background:Junctional ectopic tachycardia (JET) is caused by ectopic rhythms, originating in the atrioventricular node, typically with heart rate between 200 and 250 bpm. Herein, we present a case of fetal JET with normal fetal heart rate and a review of nine cases., Case presentation:A 32-year-old, gravida 2, para 1, woman in whom fetal JET could not be diagnosed prenatally because the fetal heart rate was within the normal range. The fetus was diagnosed with premature restriction of the foramen ovale, and a cesarean section was performed, owing to the right heart overload that was characterized by fetal ascites and abnormal fetal Doppler velocity. Postnatally, the female neonate was diagnosed with JET on a 12-lead electrocardiogram, which revealed a neonatal heart rate of 158 bpm with narrow QRS and atrioventricular dissociation. After failure to respond to amiodarone therapy, she was treated with flecainide, which controlled the JET rate from 120 to 150 bpm. Fetal tachycardia with ventriculo-atrial (VA) dissociation or 1:1 VA conduction with a shorter VA interval than that of atrioventricular reentrant tachycardia confirmed the diagnosis of fetal JET., Conclusions:JET should be suspected even in the absence of tachycardia in patients with ductus venosus and pulmonary vein retrograde flow or tricuspid and mitral regurgitation without a cardiac anomaly, as tachycardia might sometimes be intermittent in cases of JET.

Objective:To assess regional differences in reduction of the incidence of Kawasaki disease during the mitigation period for the coronavirus disease 2019 pandemic, with a hypothesis that more sparsely populated regions have fewer opportunities for human-to-human contact, resulting in a greater reduction in the incidence of Kawasaki disease., Study design:A retrospective ecological study was conducted using data from patients hospitalized for Kawasaki disease as well as infectious diseases surveillance reports in Shiga Prefecture, Japan, during 2015-2020. We defined the periods before and after the onset of pandemic as January 2015-March 2020 and as April 2020-December 2020, respectively. We compared the reductions in the incidence of Kawasaki disease among 6 administrative regions in the prefecture according to the density of the populations., Results:A total of 1290 patients with Kawasaki disease were identified. The incidence of Kawasaki disease (per 100 000 person-years) was significantly reduced after the coronavirus disease 2019 pandemic onset (period before pandemic onset, 105.6 [95% CI 99.8-111.8]; period after pandemic onset, 68.6 [95% CI 56.7-83.0]). During the period after pandemic onset, the incidence of Kawasaki disease was significantly reduced in May, compared with the corresponding period in previous years. The number of patients aged 2-4 years was significantly reduced after the pandemic onset. Notably, greater reductions in the incidence of Kawasaki disease were found in regions with lower population densities., Conclusions:Assuming that there were fewer opportunities for human-to-human contact in more sparsely populated regions during the pandemic mitigation period, our findings support the hypothesis that human-to-human contact may be associated with development of Kawasaki disease.

To determine the safety, pharmacokinetics, and immunomodulatory effects of 2-6 weeks of anakinra therapy in patients with acute Kawasaki disease with a coronary artery aneurysm (CAA).We performed a Phase I/IIa dose-escalation study of anakinra (2-11 mg/kg/day) in 22 patients with acute Kawasaki disease with CAA. We measured interleukin (IL)-1RA concentrations after the first dose and trough levels up to study week 6. Markers of inflammation and coronary artery z-scores were assessed pretreatment and at 48 hours, 2 weeks, and 6 weeks after initiation of therapy.Up to 6 weeks of anakinra (up to 11 mg/kg/day) was safe and well tolerated by the 22 participants (median age, 1.1 years), with no serious adverse events attributable to the study drug. All participants were treated with intravenous immunoglobulin (IVIG), and 20 also received infliximab (10 mg/kg) before initiation of anakinra. Serum levels of IL-6, IL-8, and tumor necrosis factor α decreased similarly in patients with Kawasaki disease treated with IVIG, infliximab, and anakinra compared with age- and sex-matched patients with Kawasaki disease treated only with IVIG and infliximab. Anakinra clearance increased with illness day at diagnosis. Simulations demonstrated that more frequent intravenous (IV) dosing may result in more sustained concentrations without significantly increasing the peak concentration compared with subcutaneous (SC) dosing.Both IV and SC anakinra are safe in infants and children with acute Kawasaki disease and CAA. IV dosing every 8-12 hours during the acute hospitalization of patients with Kawasaki disease may result in a sustained concentration while avoiding frequent SC injections. The efficacy of a short course of IV therapy during hospitalization should be studied. TRIAL REGISTRATION CLINICALTRIALS.GOV: NCT02179853.

Background: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy. Although it is associated with high morbidity and mortality, the related ion channel gene variants in children have not been fully investigated. This study aimed to elucidate the ion channel genetic landscape of LVNC and identify genotype-phenotype correlations in a large Japanese cohort. Methods: We enrolled 206 children with LVNC from 2002 to 2017 in Japan. LVNC was classified as follows: LVNC with congenital heart defects, arrhythmia, dilated phenotype, or normal function. In the enrolled patients, 182 genes associated with cardiomyopathy were screened using next-generation sequencing. Results: We identified 99 pathogenic variants in 40 genes in 87 patients. Of the pathogenic variants, 8.8% were in genes associated with channelopathies, 27% were in sarcomere genes, and 11.5% were in mitochondrial genes. Ion channel gene variants were mostly associated with the arrhythmia classification, whereas sarcomere and mitochondrial gene variants were associated with the dilated phenotype. Echocardiography revealed that the group with ion channel gene variants had almost normal LV ejection fraction and LV diastolic diameter Z scores. Fragmented QRS, old age, and an arrhythmia phenotype were the most significant risk factors for ventricular tachycardia ( P =0.165, 0.0428, and 0.0074, respectively). Moreover, the group with ion channel variants exhibited a greater risk of a higher prevalence of arrhythmias such as ventricular tachycardia, rather than congestive heart failure. Conclusions: This is the first study that focused on genotype-phenotype correlations in a large pediatric LVNC patient cohort with ion channel gene variants that were determined using next-generation sequencing. Ion channel gene variants were strongly correlated with arrhythmia phenotypes. Genetic testing and phenotype specification allow for appropriate medical management of specific LVNC targets.

Background Coronary artery aneurysms and myocarditis are well‐recognized complications of Kawasaki disease ( KD ) but no systematic evaluation of the consequences of myocarditis has been performed in the subset presenting with low ejection fraction ( EF ). We postulated that more severe myocardial inflammation as evidenced by low EF during the acute phase could lead to late myocardial fibrosis. Methods and Results We measured the carboxyterminal propeptide of procollagen type I ( PIPC ), soluble suppressor of tumorigenicity 2, galectin‐3 (Gal‐3), growth‐differentiation factor‐15, and calprotectin by ELISA in late convalescent blood samples from 16 KD patients who had an EF ≤55% on their initial echocardiogram. Results were compared with samples from sex‐ and age‐matched KD patients with initial EF >60%. In the univariate analysis, the median Gal‐3 and PIPC levels in the low EF group were significantly higher than those in the normal EF group (Gal‐3: low EF 6.216 versus normal EF 4.976 mg/dL P =0.038, PIPC : low EF 427.4 versus normal EF 265.2 mg/dL, P =0.01). In a multivariable analysis, there were significant differences for Gal‐3 and PIPC levels between the low and normal EF groups, adjusting for age, sex, and worst z score. Conclusions Convalescent KD patients with a history of low EF during the acute illness had significantly elevated levels of Gal‐3 and PIPC when compared with matched‐control KD patients with normal EF . These findings raise concern for myocardial fibrosis as a potential late sequela of the more severe myocarditis experienced by a subset of KD patients during the acute phase.

Background: The sequelae of Kawasaki disease (KD) vary widely with the greatest risk for future cardiovascular events among those who develop giant coronary artery aneurysms (CAA). We sought to define the molecular signature associated with different outcomes in pediatric and adult KD patients. Methods: Molecular profiling was conducted using mass spectrometry–based shotgun proteomics, transcriptomics, and glycomics methods on 8 pediatric KD patients at the acute, subacute, and convalescent time points. Shotgun proteomics was performed on 9 KD adults with giant CAA and matched healthy controls. Plasma calprotectin was measured by ELISA in 28 pediatric KD patients 1 year post-KD, 70 adult KD patients, and 86 healthy adult volunteers. Results: A characteristic molecular profile was seen in pediatric patients during the acute disease, which resolved at the subacute and convalescent periods in patients with no coronary artery sequelae but persisted in 2 patients who developed giant CAA. We, therefore, investigated persistence of inflammation in KD adults with giant CAA by shotgun proteomics that revealed a signature of active inflammation, immune regulation, and cell trafficking. Correlating results obtained using shotgun proteomics in the pediatric and adult KD cohorts identified elevated calprotectin levels in the plasma of patients with CAA. Investigation of expanded pediatric and adult KD cohorts revealed elevated levels of calprotectin in pediatric patients with giant CAA 1 year post-KD and in adult KD patients who developed giant CAA in childhood. Conclusions: Complex patterns of biomarkers of inflammation and cell trafficking can persist long after the acute phase of KD in patients with giant CAA. Elevated levels of plasma calprotectin months to decades after acute KD and infiltration of cells expressing S100A8 and A9 in vascular tissues suggest ongoing, subclinical inflammation. Calprotectin may serve as a biomarker to inform the management of KD patients following the acute illness.

To investigate the efficacy and safety of percutaneous transluminal balloon dilation (PTBD) for the treatment of bilateral pulmonary artery banding (bil-PAB) site stenosis.Although bil-PAB is an alternative initial treatment for high-risk neonates with hypoplastic left heart syndrome (HLHS) or critical aortic stenosis (cAS), those patients often suffer from desaturation because of progressive stenosis of the bil-PAB sites during the interstage period.We retrospectively evaluated the efficacy and safety of 11 consecutive PTBD procedures performed between 2006 and 2012 to treat bil-PAB site stenosis in four high-risk infants (three females) with HLHS or cAS.PTBD was repeated twice in two patients and three times in one patient over intervals. The mean balloon diameter (BD) and BD-to-band circumference (BC) ratio were 3.1 ± 0.5 mm and 0.31 ± 0.06, respectively. After the procedures, the mean minimum lumen diameter was dilated significantly from 1.1 ± 0.1 mm to 1.7 ± 0.3 mm (P 0.01), and the mean peripheral oxygen saturation increased significantly from 75 ± 8% to 85 ± 4% (P 0.01). All patients reached the next stage operation involving the Norwoodbidirectional Glenn or Ross procedure, after growth. No complications such as band rupture occurred.For progressive stenosis of bil-PAB sites, PTBD using a balloon size that did not exceed the BC (BD around 30% of the BC) was an effective and safe procedure.

The aim of this study was to determine whether electrocardiographic signs correlate with hemodynamics and the magnitude of the intracardiac shunt in children with ostium secundum atrial septal defects (ASD).A total of 100 ASD patients (median age, 6 years 4 months; 54 girls) underwent cardiac catheterization between August 1980 and April 2010. We retrospectively investigated the relationship between electrocardiographic signs and the pulmonary/systemic blood flow ratio (Qp/Qs) in these patients. We also compared 63 postoperative electrocardiograms with those recorded before surgery.The mean Qp/Qs ratio of the 100 patients was 2.46 ± 0.81 (range, 1.1-5.0). The Qp/Qs ratio in patients with and without right bundle branch block (RBBB) was 2.57 ± 0.82 (n = 73) and 2.15 ± 0.72 (n = 27), respectively (P = 0.016). The Qp/Qs ratio in patients with and without isolated negative T-wave was 2.85 ± 0.87 (n = 38) and 2.22 ± 0.68 (n = 62), respectively (P = 0.0003). None of the patients with low Qp/Qs ratio (Qp/Qs ratio ≤ 1.5) had both RBBB and isolated negative T-wave. The prevalence of these two signs decreased from 73.0% (n = 46) and 36.5% (n = 23) to 15.9% (n = 10) and 15.9% (n = 10) after surgical repair, respectively.RBBB and isolated negative T-wave in the precordial leads are well correlated with high Qp/Qs ratio in ASD patients.

Objective To determine the long-term outcome of systemic artery aneurysms (SAAs) after Kawasaki disease (KD). Study design We investigated the characteristics and the fate of SAAs in 20 patients using medical records and angiograms. The age of onset of KD ranged from 1 month to 20 months. The interval from the onset of KD to the latest angiogram ranged from 16 months to 24 years. The regression rate of peripheral artery aneurysm and the frequency of stenotic lesions were analyzed by the Kaplan-Meier method in 11 patients who had undergone initial angiography within 4 months. Results The mean duration of fever was 24 ± 12 days. All 20 patients had at least 1 symmetric pair of aneurysms in bilateral peripheral arteries, and 16 patients had multiple SAAs. The distributions of SAAs was as follows: brachial artery, 30; common iliac artery, 20; internal iliac artery, 21; abdominal aortic aneurysm, 7; and others, 29. The frequencies of regression of SAA and of the occurrence of stenotic lesions at 20 years after the onset of KD were 51% and 25%, respectively (n = 42). The diameter of all SAAs in the acute phase leading to stenotic lesions in the late period was >10 mm. Conclusion SAAs occurred symmetrically and were multiple in younger infants and those with severe acute vasculitis. The fate of SAAs resembles that of coronary artery aneurysms, and depends on the diameter during the acute phase. Larger SAAs can lead to stenotic lesions in the late period.