Effectiveness of two same-manufacturer intravenous immunoglobulin for Kawasaki disease.

BACKGROUND: To investigate whether two brands of intravenous immunoglobulin (IVIG) from the same manufacturer lead to varied effects when administered to patients with Kawasaki disease. (KD) METHODS: Clinical characteristics, laboratory data, IVIG response, and coronary arteries change were analyzed between two groups. RESULTS: We included 158 KD cases. The mean age at KD diagnosis was 23 ± 1.39 (range, 2-95) months. In the first IVIG course, 18 (11.4 %) patients were unresponsive. TBSF (brand T) and Privigen (brand P) were administered to 94 and 64 patients, respectively. The brand P group had a significantly longer fever (P < 0.001) and hospitalization (P = 0.007) durations after the therapy and a higher number of IVIG unresponsiveness (P = 0.016) than the brand T group. In the multivariate logistic regression analysis, a high Formosa score (≥3, Odds ratio [OR], 4.939; 95 % confidence interval [CI], 1.199-20.337; P = 0.027), high levels of CRP (≥12 mg/L, OR: 4.257,95 % CI,1.265-14.322; P = 0.019), and treatment with brand P (OR, 3.621; 95 % CI, 1.029-12.677; P = 0.045) were independent risk factors for IVIG unresponsiveness. CONCLUSIONS: Compared with brand T, brand P prolonged the fever and hospitalization durations after IVIG treatment and increased the proportion of IVIG treatment unresponsiveness, but it did not infer the coronary arteries sequelae.

Multicenter registry of multisystem inflammatory syndrome in children (MIS-C) and Paired comparison with Kawasaki disease.

OBJECTIVES: This study aimed to identify clinical characteristics to differentiate multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) in Taiwan, an island with a delayed cluster of MIS-C and a high incidence of KD. Additionally, we studied risk factors for developing severe complications in patients with MIS-C. METHODS: We conducted a retrospective, multicenter, cohort, and observational study that linked data on patients with MIS-C between May and December 2022 and patients with KD between 2019 and 2021 from 12 medical centers. Hemodynamic compromise, defined as the need for inotropic support or fluid challenge, was recorded in patients with MIS-C. We also evaluated maximal coronary Z-scores before treatment and one month after disease onset. RESULTS: A total of 83 patients with MIS-C and 466 patients with KD were recruited. A 1:1 age and gender-matched comparison of 68 MIS-C and KD pairs showed that MIS-C patients had a lower percentage of positive BCG red halos, lower leukocyte/platelet counts, more gastrointestinal symptoms, and a higher risk of hemodynamic compromise. In Taiwan, 38.6% of MIS-C patients experienced hemodynamic compromise, with presence of conjunctivitis and elevated levels of procalcitonin (>1.62 ng/mL) identified as independent risk factors. CONCLUSIONS: We identified two independent risk factors associated with hemodynamic compromise in MIS-C patients. The comparison between matched MIS-C and KD patients highlighted significant differences in clinical presentations, like BCG red halos, which may aid in the differential diagnosis of the two disease entities, especially in regions with a high incidence rate of KD.

Kawasaki disease in children with Bacillus Calmette-Guérin scar reactivity: Focus on coronary outcomes.

BACKGROUND: /Purpose: Reactivity at the Bacillus Calmette-Guérin (BCG) scar is a pathognomonic feature of Kawasaki disease (KD). However, its value in predicting KD outcomes has not been emphasized. This study explored the clinical significance of BCG scar redness with respect to coronary artery outcomes. METHODS: This retrospective study collected data on children with KD from 13 hospitals in Taiwan during 2019-2021. Children with KD were categorized into four groups based on the KD type and BCG scar reactivity. Risk factors of coronary artery abnormalities (CAA) were analyzed in all groups. RESULTS: BCG scar redness occurred in 49% of 388 children with KD. BCG scar redness was associated with younger age, early intravenous immunoglobulin (IVIG) treatment, hypoalbuminemia, and CAA at the first echocardiogram (p < 0.01). BCG scar redness (RR 0.56) and pyuria (RR 2.61) were independent predictors of any CAA within 1 month (p < 0.05). Moreover, pyuria (RR 5.85, p < 0.05) in children with complete KD plus BCG scar redness was associated with CAA at 2-3 months; first IVIG resistance (RR 15.2) and neutrophil levels ≥80% (RR 8.37) in children with complete KD plus BCG scar non-redness were associated with CAA at 2-3 months (p < 0.05). We failed to detect any significant risk factors of CAA at 2-3 months in children with incomplete KD. CONCLUSION: BCG scar reactivity contributes to diverse clinical features in KD. It can be effectively applied to determine the risk factors of any CAA within 1 month and CAA at 2-3 months.

Zero-fluoroscopy ablation of left-sided arrhythmia substrates in children - Mid-term safety and feasibility study from transaortic approach.

BACKGROUND: A widely used method of treating left-sided arrhythmia substrates in children is retrograde transaortic ablation under fluoroscopic guidance. However, the feasibility, safety, and efficacy of this approach under zero fluoroscopy (ZF) guidance, especially the mid-term safety of anatomy and function of aortic valves, have yet to be proven. METHODS: All consecutive patients who received ablation of left-sided arrhythmias between January 2012 and June 2020 and below 20 years-old were enrolled. The study group submitted to 55 ZF-guided procedures using cardiac mapping system (EnSite Precision), whereas 49 procedures were performed under fluoroscopic guidance in the control group. Echocardiographic studies took place before and 6-months after ablative procedures. RESULTS: One-hundred-and-two patients (male, 66; female, 36) underwent a total of 104 ablative procedures. Mean procedural durations were 83.9 ± 44.4 min in the study group and 64.8 ± 29.1 min in the control group, respectively (p = .01; the 95% confidence interval, -33.57 to -4.63). Corresponding fluoroscopic times were .5 ± 2.2 min and 24.7 ± 13.9 min (p < .001; the 95% confidence interval, 20.15 to 28.22). ZF may be reasonably applied after a learning curve of 20 cases. Immediate procedural success and recurrence rates were similar in each groups. There was no detectable progression of aortic regurgitation in any of the patients during serial follow-up of echocardiography. CONCLUSION: ZF-guided retrograde transaortic ablation of left-sided arrhythmia substrates proved safe in children at midterm follow-up, reducing radiation exposure significantly within a learning curve of <20 cases.

Acute Outcomes for Cryoablation in Pediatric Patients with Perinodal Tachyarrhythmia: Single Center Report.

BACKGROUND: Cryoablation is an alternative treatment for atrioventricular nodal reentrant tachycardia (AVNRT) and right anteroseptal and midseptal accessory pathways (APs) with a low complication rate. A high recurrence rate is still a concern in pediatric patients. METHODS: From February 2015 to March 2017, all consecutive patients who underwent cryoablation for supraventricular tachycardia were included in this study. The demographic and clinical data of the patients were reviewed. RESULTS: Fifty-two patients (AVNRT 43, anteroseptal and midseptal AP 9) were enrolled, including 24 males and 28 females. The median age at the time of the procedure was 15.6 years. For patients with AVNRT, 34 (79.1%) had the typical form, 5 had the atypical form (11.6%), and another 4 had both forms. For AP, four patients had right midseptal and 5 had right anteroseptal APs. The median total procedure time was 114 min (range 69-331 min), and the median fluoroscopy time was 25.9 min (range 9.2-99.6 min). After a median 6 attempts of cryomapping and 3 of cryoablation, the arrhythmia substrate was successfully ablated in 51 of 52 patients (98.1%). Ten developed transient second degree atrioventricular (AV) block and one developed transient third degree AV block, but none had permanent AV block or other complications. After a mean follow-up of 1.95 ± 0.54 years (range 1.1-2.86 years), there were three cases of recurrence (5.9%). The mean number of cryoablations decreased from 6.6 ± 6.4 (early group) to 3.1 ± 2.6 (late group) (p = 0.01) after a 1-year learning period. CONCLUSIONS: Cryoablation for AVNRT and anteroseptal and midseptal APs in pediatric and adolescent patients is safe and effective.

Zero Fluoroscopy During Ablation of Right-Sided Supraventricular Tachycardia Substrates in a Pediatric Population - Initial Experience in Taiwan.

BACKGROUND: Zero fluoroscopy during ablation of arrhythmias has been introduced to reduce radiation exposure. However, the safety, feasibility and efficacy of the technique in pediatric populations have yet to be delineated. OBJECTIVES: To investigate the safety, feasibility and effectiveness of zero fluoroscopic-guided transcatheter ablation of right-sided supraventricular tachycardia substrates in a pediatric population. METHODS: Patients < 20 years of age who received ablation of right-sided accessory pathway-mediated arrhythmia and atrioventricular nodal reentrant tachycardia at our hospital between January 2014 and July 2018 were enrolled, and their medical records were reviewed. Patients undergoing ablations with conventional fluoroscopic guidance were enrolled as the control group, and those undergoing ablations with zero fluoroscopic guidance were enrolled as the study group. Repaired or complex congenital heart disease patients were excluded. RESULTS: One hundred and two patients (55 male; 47 female) received a total of 109 ablation procedures: 68 procedures in the control group and 41 in the study group. The mean procedure duration was 104.7 ± 65.1 minutes in the control group and 98.6 ± 57.6 minutes in the study group (p = 0.62). The mean fluoroscopy time was 30.9 ± 23.9 minutes in the control group, while all procedures in the study group were performed without fluoroscopy (p < 0.001). In subgroup analysis, the results were similar. Acute procedural success rates were high in both groups (98.5% vs. 97.6%, p = 1.0). The recurrence rate was 11.5% (7/61) in the control group and 7.5% (3/40) in the study group (p = 0.78) at mid-term follow-up. CONCLUSIONS: Using the zero fluoroscopy technique during pediatric ablation procedures for right-sided supraventricular tachycardia substrates is safe and significantly reduces radiation exposure.

Combination of slow pathway late activation maps and voltage gradient maps in guidance of atrioventricular nodal reentrant tachycardia cryoablation.

BACKGROUND: The optimal strategy for electroanatomic mapping-guided cryoablation of atrioventricular nodal reentry tachycardia (AVNRT) remains unclear. OBJECTIVE: The purpose of this study was to investigate the effectiveness of slow pathway late activation mapping (SPLAM) and voltage gradient mapping for AVNRT cryoablation. METHODS: From June 2020 to February 2022, all consecutive patients with AVNRT underwent SPLAM to define the wave collision point and voltage gradient mapping to define the low-voltage bridge (LVB). Conventional procedures performed from August 2018 to May 2020 served as control. RESULTS: The study and control groups comprised 36 patients (age 16.5 ± 8.2 years) and 37 patients (age 15.5 ± 7.3 years), respectively. Total procedural times were comparable, and acute success rates were 100% in both groups. Compared to controls, the number of cryomapping attempts (median 3 vs 5; P = .012) and cryoablation applications (median 1 vs 2; P <.001) were significantly lower in the study group. At median follow-up of 14.6 and 18.3 months, recurrence rates were 5.6% (2 patients) and 10.8% (4 patients) in the study and control groups (P = .402), respectively. Mapping of the Koch triangle took 11.8 ± 3.6 minutes, during which 1562 ± 581 points were collected. In SPLAM, wave collision points were defined and compatible with the final successful lesion sites in all patients, including those with multiple slow pathways. LVB could not be defined in 6 patients (16.7%), and LVB was not compatible with the final successful lesion in another 6 (16.7%). CONCLUSION: For AVNRT cryoablation, SPLAM could effectively guide the localization of slow pathway ablation sites and was particularly beneficial in patients with multiple slow pathways.

Cross-protective humoral immunity to coronaviruses from SARS coronavirus 2-naïve sera of children with Kawasaki disease.

OBJECTIVES: SARS coronavirus 2 (SARS-CoV-2)-associated multi-system inflammatory syndrome in children indicates that viruses can trigger a Kawasaki disease (KD)-like hyperinflammation. A plausible hypothesis was that coronavirus-specific 'holes' in humoral immunity could cause both diseases. METHODS: To determine whether SARS-CoV-2-naïve patients with KD have inferior humoral immunity for the novel coronavirus, sera of children with KD and control children from year 2015 to 2021 were subjected to ELISA, microwestern, and neutralization assays to evaluate the capabilities in recognizing the receptor-binding domain of SARS-CoV-2, spotting spike proteins of three respiratory syndromic coronaviruses, and blocking SARS-CoV-2 from binding to angiotensin-converting enzyme 2 receptors in vitro, respectively. RESULTS: 29 patients with KD before 2019, 74 patients with KD in 2019 or 2020, 54 non-febrile controls, and 24 febrile controls were included in the study. SARS-CoV-2 was recognized on ELISA for both patients with KD in 2016 and those with KD in 2020. Microwestern demonstrated cross-reactive IgG in an all-or-none manner towards three spike proteins of syndromic coronaviruses regardless of sample year or KD status. The ratio between the sera that recognized all spike proteins and those that recognized none (51 vs. 47) was significantly higher from patients with KD than from non-febrile controls (17 vs. 32; p 0.047) but not from febrile controls (13 vs. 11; p 0.85). Most positive sera (12 of 17 controls, 5 of 8 patients with KD before 2019, and 28 of 33 patients with KD in 2019 or 2020) offered protection comparable to low-titre sera from the WHO reference panel. DISCUSSION: Humoral immunity of SARS-CoV-2-naïve children with KD was not inferior to that of controls in offering cross-protection against the novel coronavirus.

Left ventricular outflow tract obstruction in complete transposition of the great arteries--echocardiography criteria for surgical strategies.

BACKGROUND: The arterial switch operation (ASO) may be infeasible in patients with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS AND RESULTS: Patients with LVOTO were identified among the 229 patients with TGA repaired between 1991 and 2006. Dynamic LVOTO was defined as LVOTO without LVOT abnormalities requiring resection (group I, receiving ASO). Fixed LVOTO was defined as LVOTO with LVOT abnormalities that could (group IIa, receiving ASO) or could not be resected (group IIb, receiving operations other than ASO). Preoperative echocardiography parameters were compared to identify criteria predicting the surgical findings of LVOTO. LVOTO was found in 63 patients (27.5%): 39 with dynamic and 24 with fixed (14 group IIa and 10 IIb) LVOTO. Late, severe neo-aortic regurgitation occurred more frequently in group IIa patients. Echocardiography evidence of isolated subvalvular stenosis, pulmonary to aortic valvular annulus ratio >0.98 or LVOT pressure gradient <22 mmHg predicted dynamic LVOTO (sensitivity 86.3%, specificity 94.7%), whereas that of an annulus ratio <0.85 and a pressure gradient >35 mmHg predicted fixed and non-resectable LVOTO (sensitivity 100%, specificity 100%). CONCLUSIONS: Approximately one-fourth of TGA patients had LVOTO. Echocardiography criteria identified those with dynamic or fixed but resectable (ASO) LVOTO with satisfactory sensitivity and specificity.

Dilated cardiomyopathy after long-term right ventricular apical pacing in children with complete atrioventricular block: role of setting of ventricular pacing.

BACKGROUND: Pediatric patients paced for complete atrioventricular block (CAVB) are at risk of developing dilated cardiomyopathy (DCM). We sought to explore the role played by the setting of ventricular pacing. METHODS AND RESULTS: A total of 35 patients <18 years of age at diagnosis were enrolled. Twenty-eight (80%) received pacemakers after a mean follow-up of 10 years, and most were paced from right ventricular (RV) apex (n = 23). None of the 7 patients without pacemakers developed DCM, whereas 8 (35%) paced from RV apex had DCM 2.6 +/- 2.2 years after pacing. The percentage of ventricular pacing was >90% in all patients. Multivariate analysis revealed that the cumulative Z score of lower ventricular rate setting was the single most important risk factor for DCM (HR, 3.14; 95% CI, 1.07-9.19; P = .037). Subgroup analysis in patients with VVI/VVIR modes revealed an even stronger predictive value of the cumulative Z score of lower ventricular rate setting (HR, 9.12; 95% CI, 1.53-54.24; P = .015). CONCLUSIONS: Higher setting of the lower ventricular rate, though still within the age-appropriate range, was associated with increased risk of developing DCM in pediatric patients with CAVB and chronic RV apical pacing.

Special electrophysiological characteristics of pediatric idiopathic ventricular tachycardia.

BACKGROUND: Idiopathic ventricular tachycardia (VT) is an important pediatric arrhythmia but relevant studies in Asian cohort are rare. We determined the electrophysiological characteristics and outcomes of this rhythm disturbance. METHODS: From an institutional database, patients aged <18years without structural heart disease who have sustained monomorphic VT (longer than 30s) during 1995-2016 were enrolled. RESULTS: We enrolled 57 idiopathic VT patients (male:female=31:26). The initial presentation included palpitation (64.9%) and shock (12.3%). The electrocardiography revealed relatively short QRS duration (123±18ms) and absence of ventriculoatrial dissociation in 33.3% of the patients, causing initial misdiagnosis in 29.8% of these patients. Patients with a VT onset age of <10years were more likely to have a left bundle branch block morphology (52.2% vs. 11.8%, p=0.001) and spontaneous recovery than those with a VT onset age of ≥10years. An electrophysiology study in 44 patients revealed that the most common arrhythmia focus was the left ventricular septal area (78%), called fascicular VT. Radiofrequency ablation showed a satisfactory success rate (90%) and fair recurrence rate (14.3%). Tachycardia-induced cardiomyopathy was noted in 14% of the VT patients and cardiac function returned to normal in 1-18months (median, 3months) after successful ablation. CONCLUSIONS: In an Asian pediatric population, VT has distinct electrophysiological characteristics including dominant left ventricular origin, narrower QRS duration, and more symptomatic presentation. Transcatheter ablation is effective against VT, and the associated cardiomyopathy often resolves rapidly after successful ablation.

Primary ventricular tachycardia in paediatric population in a tertiary centre.

OBJECTIVE: To delineate the outcome of ventricular tachycardia (VT) in the paediatric population. METHODS: Patients who developed sustained VT between the ages of 0 and 18 years in a referral centre from 1991 to 2015 were enrolled. RESULTS: A total of 116 patients (67 male/49 female) had documented VT, and 53 (46%) had associated heart disease, namely cardiomyopathy in 20 (17%), structural heart disease in 19 (16%) and channelopathy in 14 (12%), and some of them presented with two types of associated heart disease. Idiopathic VT (63 patients), which presents without associated heart disease, was the most common type. Forty-one patients received catheter ablation, with 37 being successful (90%) and 6 of 37 recurrence (16%). None of the patients died during the 5.8±5.9 year follow-up. VT with cardiomyopathy was associated with the highest mortality rate, particularly in those with hypertrophic and restrictive cardiomyopathy. Among 16 patients initially presenting VT and heart failure, seven exhibited improved heart function after VT control, which could be predicted by benign onset symptoms, monomorphic QRS morphology and the presentation of VT at the initial diagnosis of cardiomyopathy. VT associated with structural heart disease was also associated with a high risk of mortality, but this risk decreased after aggressive intervention in the recent years. VT with channelopathy can be often controlled with medication, except for those with prenatal onset. CONCLUSIONS: Although VT may carry high mortality when associated with structural anomaly or cardiomyopathy, VT presenting to tertiary referral centre often has a favourable outcome after prompt intervention.

Electrophysiology Study for Complex Supraventricular Tachycardia in Congenital Heart Disease Patients With Single-Ventricle Physiology.

BACKGROUND: Supraventricular tachycardia (SVT) is common in complex congenital heart disease (CCHD) patients with single-ventricle physiology and may cause hemodynamic deterioration. We reported the outcomes of catheter ablation for such complex SVT in these single-ventricle CCHD patients. METHODS AND RESULTS: Patients with single-ventricle physiology (defined as CCHD patients) who received electrophysiology studies and catheter ablation between 1995 and 2015 were studied. We enrolled 30 CCHD patients (18 with right atrial isomerism, 5 with left atrial isomerism, and 7 with other CCHDs; 17 male, 13 female). The age of onset of clinical SVT was 6.7 years (±4.7 years). Electrophysiology studies and ablation were performed at age 7.1 years (±3.9 years); body weight was 20.7 kg (±10.0 kg). Twin atrioventricular nodes were present in 60% of patients (right atrial isomerism, 72.2%; left atrial isomerism, 40%; other CCHDs, 42.9%). Manifested preexcitation was noted in 10% of patients. SVT was induced in 21 patients. Twin atrioventricular nodal reentrant tachycardia was the most common (57.1%), followed by atrioventricular reentrant tachycardia (28.6%), junctional tachycardia (14.3%), and atrioventricular nodal reentrant tachycardia (9.5%). Multiple arrhythmias were common (33.3%), particularly in patients with atrioventricular reentrant tachycardia (50%). Ablation successfully eliminated SVT in 12 of 14 patients (85.7%), with a recurrence rate of 16.7% during 6 years of follow-up. CONCLUSIONS: Transcatheter ablation of complex SVT substrates, including minor atrioventricular node of twin atrioventricular nodal reentrant tachycardia, accessory pathways of atrioventricular reentrant tachycardia, and a slow pathway of atrioventricular nodal reentrant tachycardia, is effective in CCHD patients. The limitations are limited vascular access and the risk of atrioventricular block.

Arterial switch operation for transposition of the great arteries: experience from 2000-2002 in Taiwan.

Arterial switch operation (ASO) is considered the procedure of choice for transposition of great arteries (TGA). The results and long-term prognosis improved with recent advances in perioperative management. We herein analyze the clinical outcome of patients undergoing ASO at our institution during the past 3 years. From 2000 to 2002, 44 patients (30 male and 14 female) of TGA received ASO. Age at operation varied from 4 days to 6.6 years (median 14 days) with body weight ranged from 2.25 kg to 18.1 kg (median 3.3 kg). Palliative procedure prior to ASO was performed in 8 patients (18.8%). Normal coronary artery pattern was found in 28 patients (63.6%). The early mortality was 11% (5/44). Only associated ventricular septal defect (VSD) was a significant predictor for operative mortality (p=0.012). With a follow-up ranged from 11 to 44 months, the gradient of neo-pulmonary artery stenosis was 16.5 +/- 18.2 mmHg. Four patients (10%) received balloon dilatation and the other three (7.7%) underwent reoperation. The gradient of neo-aortic stenosis was 16.5 +/- 18.2 mmHg that needed to be dilated in three patients (7.7%). The probability free from reintervention was 73% at the 3rd postoperative year. One patient had moderate degree of pulmonary valve regurgitation and six had moderate neo-aortic valve regurgitation. In conclusion, the ASO can be performed in infants with satisfactory results, even in those with a body weight less than 2.5 kg. Only associated VSD was shown to be a risk factor.