Extracorporeal Membrane Oxygenation in Children With COVID-19: A Systematic Review and Meta-Analysis.

OBJECTIVES: The indication, complications, and outcomes of extracorporeal membrane oxygenation (ECMO) in children with COVID-19-related illnesses remain unelucidated. Our study aimed to investigate the characteristics and outcomes of ECMO in children with COVID-19-related illnesses. DATA SOURCES: We searched PubMed and EMBASE databases in March 2022. STUDY SELECTION: We retrieved all studies involving children (age ≤ 18 yr) with COVID-19-related illnesses who received ECMO. DATA EXTRACTION: Two authors independently extracted data and assessed the risk of bias. Mortality, successful weaning rate, and complications while on ECMO were synthesized by a one-group meta-analysis using a random-effect model. Meta-regression was performed to explore the risk factors for mortality. DATA SYNTHESIS: We included 18 observational studies, four case series, and 22 case reports involving 110 children with COVID-19-related illnesses receiving ECMO. The median age was 8 years (range, 10 d to 18 yr), and the median body mass index was 21.4 kg/m 2 (range, 12.3-56.0 kg/m 2 ). The most common comorbidities were obesity (11% [7/63]) and congenital heart disease (11% [7/63]), whereas 48% (30/63) were previously healthy. The most common indications for ECMO were multisystem inflammatory syndrome in children (52% [47/90]) and severe acute respiratory distress syndrome (40% [36/90]). Seventy-one percent (56/79) received venoarterial-ECMO. The median ECMO runtime was 6 days (range, 3-51 d) for venoarterial ECMO and 11 days (range, 3-71 d) for venovenous ECMO. The mortality was 26.6% (95% CI, 15.9-40.9), and the successful weaning rate was 77.0% (95% CI, 55.4-90.1). Complications were seen in 37.0% (95% CI, 23.1-53.5) while on ECMO, including stroke, acute kidney injury, pulmonary edema, and thromboembolism. Corticosteroids and IV immunoglobulin therapies were associated with lower mortality. CONCLUSIONS: The mortality of children on ECMO for COVID-19 was relatively low. This invasive treatment can be considered as a treatment option for critically ill children with COVID-19.

Longitudinal Cardiac Outcomes of Multisystem Inflammatory Syndrome in Children: A Systematic Review and Meta-Analysis.

There is a paucity of longitudinal data on cardiac outcomes in multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. We aimed to investigate the longitudinal cardiovascular outcomes in MIS-C. PubMed and EMBASE were searched through May 2022. Observational studies were included, reporting mid-term (≥ 3 months) outcomes in children (aged < 21) with MIS-C. Data were extracted by two researchers. Longitudinal outcomes were synthesized by a one-group meta-analysis using a random-effects model. Eleven studies with a follow-up period (3 months to 1 year) were identified, including 547 MIS-C patients. The mortality was 2.5% (95% CI 1.3-4.9). The majority of left ventricular (LV) systolic dysfunction present in 46.8% (95% CI 32.7-61.3) in the acute phase resolved by 3 months, and the prevalence of LV systolic dysfunction was 1.7% (95% CI 0.5-5.7) and 2.1% (95% CI 0.8-5.4) at 3 month and 6 month follow-up, respectively. Additionally, the persistent LV systolic dysfunction in the small population was mild. However, coronary abnormalities such as coronary artery dilatation or aneurysms, seen in 23.7% (95% CI 17.7-31.1) at baseline, persisted in 4.7% (95% CI 1.5-14.3) at 3 months and 5.2% (95% CI 3.0-8.9) at 6 months. Mitral regurgitation (MR), which was observed in 56.6% (95% CI 27.7-81.6) at baseline, also persisted in 7.5% at 6 months. In conclusion, our study demonstrated largely favorable cardiac outcomes, suggesting resolution of LV systolic dysfunction in the majority of cases. However, coronary abnormalities and MR persisted in a subset of patients at mid-term follow-up.

Pulmonary vasodilator therapies in pulmonary arterial hypertension associated with CHD: a systematic review and network meta-analysis.

The optimal treatment strategy using pulmonary vasodilators in pulmonary arterial hypertension associated with CHD (PAH-CHD) remains controversial. We aimed to compare the efficacy and safety of pulmonary vasodilators in PAH-CHD. PubMed and EMBASE databases were searched through May 2022 and a network meta-analysis was conducted. The primary outcomes were mean difference of changes in 6-minute walk distance, NYHA functional class, and N-terminal pro-brain natriuretic peptide. The secondary outcomes included pulmonary vascular resistance, mean pulmonary arterial pressure, and resting oxygen saturation. We identified 14 studies, yielding 807 patients with PAH-CHD. Bosentan and sildenafil were associated with a significant increase in 6-minute walk distance from baseline compared with placebo (MD 48.92 m, 95% CI 0.32 to 97.55 and MD 59.70 m, 95% CI 0.88 to 118.53, respectively). Bosentan, sildenafil, and combination of bosentan and sildenafil were associated with significant improvement in NYHA functional class compared with placebo (MD -0.33, 95% CI -0.51 to -0.14, MD -0.58, 95% CI -0.75 to -0.22 and MD -0.62, 95% CI -0.92 to -0.31, respectively). Bosentan and sildenafil were also associated with significant improvements in secondary outcomes. These findings were largely confirmed in the subgroup analysis. Various adverse events were reported; however, serious adverse event rates were relatively low (4.8-8.7%), including right heart failure, acute kidney injury, respiratory failure, hypotension, and discontinuation of pulmonary vasodilators. In conclusion, bosentan and sildenafil were the most effective in improving prognostic risk factor such as 6-minute walk distance and NYHA class. Overall, pulmonary vasodilators were well tolerated in PAH-CHD.

Control of Heart Rate in Infant and Child Tachyarrhythmia With Reduced Cardiac Function Using Landiolol (HEARTFUL)　- Results of a Prospective, Multicenter, Uncontrolled Clinical Study.

BACKGROUND: The prospective Control of HEART rate in inFant and child tachyarrhythmia with reduced cardiac function Using Landiolol (HEARTFUL) study investigated the effectiveness and safety of landiolol, a short-acting ß1 selective blocker, in children.Methods and Results: Twenty-five inpatients aged ≥3 months to <15 years who developed supraventricular tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, and inappropriate sinus tachycardia) were treated with landiolol. The primary endpoint, the percent of patients with a reduction in heart rate ≥20% from the initial rate of tachycardia, or termination of tachycardia at 2 h after starting landiolol, was achieved in 12/25 patients (48.0%; 95% CI 28.4-67.6), which exceeded the predetermined threshold (38.0%). At 2 h after starting landiolol administration, heart rate had decreased by ≥20% in 45.8% (11/24) and recovery to sinus rhythm was achieved in 40.0% (6/15) of the patients. Adverse reactions (ARs) occurred in 24.0% (6/25) of patients, and the study was discontinued in 4.0% (1/25) of the patients; however, none of these ARs were considered serious. The most common AR was hypotension (20.0% [5/25] of patients). CONCLUSIONS: The HEARTFUL study has demonstrated the efficacy of landiolol, by reducing heart rate or terminating tachycardia, in pediatric patients with supraventricular tachyarrhythmias. Although serious ARs and concerns were not identified in this study, physicians should be always cautious of circulatory collapse due to hypotension.

Ablation characteristics and incidence of steam pops with a novel, surface temperature-controlled ablation system in an ex vivo experimental model.

BACKGROUND: A novel irrigation catheter (QDOT MICRO™) has been introduced, which enables a surface temperature-controlled ablation combined with tip cooling. However, the detailed description of its complex behavior and effect on the incidence of pops and lesion formation remains elusive. This study aimed to systematically investigate the ablation characteristics, feedback behavior, and incidence of steam pops in a simplified ex vivo swine model. METHODS: Using swine ventricular tissue perfused with saline at 37°C, we systematically created lesions with 4×3 combinations of the wattage (20, 30, 40, and 50 W) and contact force (CF, 10, 30, and 50 g). Ablation was continued for either 120 s or until a steam pop occurred and repeated 10 times with each setting. The lesion geometry, ablation index, feedback dynamics, and conditions underlying the steam pops were measured and analyzed. RESULTS: Steam pops occurred particularly frequently in combinations of a low CF and high power (10 g vs. 30 g+50 g [p < .0001]; 40 W+50 W vs. 20 W+30 W [p < .0001]). Failure to activate a feedback process was associated with a 5.1 times higher incidence of steam pops (21/109 vs.11/11, [95% CI 3.499-7.716], p < .0001). The wattage feedback was particularly evident with a high CF (30 and 50 g) and high initial wattage (40 and 50 W). The average delivered wattage at 27 W predicted the occurrence of steam pops. CONCLUSION: The temperature-controlled ablation with the QDOT MICRO™ demonstrated a complex feedback behavior, which contributed to a reduced incidence of steam pops and prolonged lead time to the pops.

Efficacy of a phosphodiesterase type 5 inhibitor on the pulmonary artery index and postoperative hemodynamics after a Fontan operation.

There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation. We divided the patients into two groups according to the change in the pulmonary artery index before and after the Fontan operation: the increased pulmonary artery index group (n = 51) and decreased pulmonary artery index group (n = 43). The pulmonary artery index after the Fontan operation was significantly higher in the increased pulmonary artery index group than decreased pulmonary artery index group (p < 0.0001). The central venous pressure (p < 0.0001) and pulmonary vascular resistance (p = 0.007) were significantly lower in the increased pulmonary artery index group. The mixed venous oxygen saturation was significantly higher in the increased pulmonary artery index group (p < 0.0001). The pulmonary artery index was more increased in the patients who were administered a phosphodiesterase type 5 inhibitor than in those who were not (p = 0.03). The change in the pulmonary artery index reflected the hemodynamics after a Fontan operation during the short term. In that respect, phosphodiesterase type 5 inhibitors might be involved in the process.

Successful Ablation of a Biatrial Tachycardia from the Superior Vena Cava after a Senning Operation for Complete Transposition of the Great Arteries.

The Senning operation used to be widely performed for an intracardiac repair in a complete transposition of the great arteries. During the long-term follow-up, supraventricular tachycardia (SVT) is often observed because of the complex suture lines. The typical mechanism of a Senning-related SVT is cavo-tricuspid isthmus-dependent atrial flutter. On rare occasions, complex SVTs (e.g., biatrial tachycardia (BiAT)) whose diagnosis and treatment are challenging, may occur. We report a rare case of a BiAT following a Senning operation that was successfully ablated from the superior vena cava, and the local activation time histogram module (CARTO3 V7 module [Biosense Webster, Irvine, CA, USA]) was crucial for analyzing the complex circuit.

Characteristics and optimal ablation settings of a novel, contact-force sensing and local impedance-enabled catheter in an ex vivo perfused swine ventricle model.

BACKGROUND: Local impedance (LI) has emerged as a new technology that informs on electrical catheter-tissue coupling during radiofrequency (RF) ablation. Recently, IntellaNav StablePoint, a novel LI-enabled catheter that equips contact force (CF) sensing, has been introduced. Although StablePoint and its predecessor IntellaNav MiFi OI share the common technology that reports LI, distinct mechanics for LI sensing between the two products raise a concern that the LI-RF lesion formation relationship may differ. METHODS: In an ex vivo swine cardiac tissue model, we investigated the initial level and range of a reduction in LI during a 60-s RF ablation and the resultant lesion characteristics at nine combinations of three energy power (30, 40, and 50 W) and CF (10, 30, and 50 g) steps. Correlations and interactions between CF, LI, wattage, and formed lesions were analyzed. Incidence of achieving LI drop plateau and that of a steam pop were also determined. RESULTS: Positive correlations existed between CF and initial LI, CF and absolute/relative LI drop, CF and lesion volume, and LI drop and lesion volume. At the same LI drop, wattage-dependent gain in lesion volume was observed. Steam pops occurred in all CF steps and the prevalence was highest at 50 W. LI drop predicted a steam pop with a cutoff value at 89Ω. CONCLUSION: In StablePoint, wattage crucially affects LI drop and lesion volume. Because 30 W ablation may by underpowered for intramural lesion formation and 50 W often resulted in a steam pop, 40 W appears to achieve the balance between the safety and efficacy.

The optimal ablation setting for a local impedance guided catheter in an in vitro experimental model.

BACKGROUND: The local impedance (LI) reflects the electrical catheter-tissue coupling and correlates with the local tissue temperature. However, there have been few clinical studies showing the recommended method for LI monitoring catheters. This study aimed to investigate the optimal ablation setting for this catheter in an in vitro experimental model. METHODS: LI monitoring catheters were used in an excised swine heart experimental model. The tissue contact force (CF) was directly monitored from an external weight scale. Radiofrequency ablation was performed with a combination of various energy power settings (30, 40, and 50 W), and various CFs (10, 30, and 50 g) for 60 s. The correlation between the LI-related indexes, power, and CF with the lesion formation was statistically analyzed. RESULTS: A positive correlation between the LI or lesion formation and CF was observed under all powers. Although the LI drop always correlated with the maximum lesion depth, lesion diameter, and lesion volume, the coefficient of the correlation value was lower under a high CF (lesion depth, diameter, and volume; 10 g, r = 0.8064, r = 0.8389, r = 0.8477; 30 g, r = 0.7590, r = 0.8063, r = 0.8060; 50 g r = 0.5555, r = 0.5701, and r = 0.5678, respectively). Steam pops occurred only under a 50 W ablation and the LI drop cutoff value for steam pops was 46 Ω. CONCLUSION: The same LI drop did not always lead to the same lesion size when the CF differed. Monitoring the LI and not exceeding 46 Ω would be useful for a safe ablation.

Ultrahigh density atrio-ventricular dual-chamber mapping as a next generation tool for ablation of accessory pathways.

INTRODUCTION: Detailed three-dimensional (3D) mapping has been useful for effective radiofrequency catheter ablation. The Rhythmia system can create atrio-ventricular dual-chamber mapping, which reveals the atrial and ventricular potentials all at once in the same map. The aim of this study was to investigate the utility of mapping the atrium and ventricle simultaneously with a high-density 3D mapping system for the ablation of accessory pathways (AP). METHODS: From July 2015 to August 2020, 111 patients underwent ablation of APs. Dual-chamber maps were created in 50 patients (median age 15 [10-54], 32 male [64.0%]), while 61 patients underwent radiofrequency (RF) ablation with conventional single-chamber 3D maps. The background characteristics and procedural details were compared between the dual-chamber mapping group and the conventional single-chamber mapping group. RESULTS: The number of RF applications (median [IQR]; 1.0 [1.0-3.0] vs. 3.0 [1.0-6.0], p = .0023), RF time (median [IQR], s; 9.2 [2.0-95.7] vs. 95.6 [4.1-248.7], p = .0107), and RF energy (median [IQR], J; 248.4 [58.7-3328.2] vs. 2867.6 [134.2-7728.4], p = .0115) were significantly lower in the dual-chamber group. The fluoroscopy time (median [IQR], min; 19.9 [14.2-26.1] vs. 26.5 [17.7-43.4], p = .0025) and fluoroscopy dose (median [IQR], mGy; 52.5 [31.3-146.0] vs. 119.0 [43.7-213.5], p = .0249) were also significantly lower in the dual-chamber than single-chamber mapping group. CONCLUSION: The dual-chamber mapping was useful for ablating accessory pathways and reducing the number of RF applications, total RF energy, and radiation exposure as compared with traditional mapping techniques.

Novel electrocardiographic criteria for short QT syndrome in children and adolescents.

AIMS: Although shortening of the corrected QT interval (QTc) is a key finding in the diagnosis of short QT syndrome (SQTS), there may be overlap of the QTc between SQTS patients and normal subjects in childhood and adolescence. We aimed to investigate electrocardiographic findings for differentiation of SQTS patients. METHODS AND RESULTS: The SQTS group comprised 34 SQTS patients <20 years old, including 9 from our institutions and 25 from previous reports. The control group comprised 61 apparently healthy subjects with an QTc of <360 ms who were selected from 13 314 participants in a school-based screening programme. We compared electrocardiographic findings, including QT and Jpoint-Tpeak intervals (QT and J-Tpeak, respectively), those corrected by using the Bazett's and Fridericia's formulae (cB and cF, respectively) and early repolarization (ER) between the groups. QT, QTc by using Bazett's formula (QTcB), QTc by using Fridericia's formula (QTcF), J-Tpeak, J-Tpeak cB, and J-Tpeak cF were significantly shorter in the SQTS group than in the control group. On receiver operating characteristic curve analysis, the area under the curve (AUC) was largest for QTcB (0.888) among QT, QTcB, and QTcF, with a cut-off value of 316 ms (sensitivity: 79.4% and specificity: 96.7%). The AUC was largest for J-Tpeak cB (0.848) among J-Tpeak, J-Tpeak cB, and J-Tpeak cF, with a cut-off value of 181 ms (sensitivity: 80.8% and specificity: 91.8%). Early repolarization was found more frequently in the SQTS group than in the control group (67% vs. 23%, P = 0.001). CONCLUSION: A QTcB <316 ms, J-Tpeak cB < 181 ms, and the presence of ER may indicate SQTS patients in childhood and adolescence.

Electrocardiographic Diagnosis of Hypertrophic Cardiomyopathy in the Pre- and Post-Diagnostic Phases in Children and Adolescents.

BACKGROUND: The usefulness of electrocardiographic (ECG) voltage criteria for diagnosing hypertrophic cardiomyopathy (HCM) in pediatric patients is poorly defined.Methods and Results:ECGs at the 1st grade (mean [±SD] age 6.6±0.3 years) were available for 11 patients diagnosed with HCM at around the 7th grade (13.2±0.3 years). ECGs were available for another 64 patients diagnosed with HCM in the 1st (n=15), 7th (n=32), and 10th (n=17) grades. Fifty-one voltage criteria were developed by grade and sex using 62,841 ECGs from the general population. Voltage criteria were set at the 99.95th percentile (1/2,000) point based on the estimated prevalence of childhood HCM (2.9 per 100,000 [1/34,483]) to decrease false negatives. Conventional criteria were from guidelines for school-aged children in Japan. Of 11 patients before diagnosis, 2 satisfied conventional criteria in 1st grade; 5 (56%) of the remaining 9 patients fulfilled 2 voltage criteria (R wave in limb-lead I [RI]+S wave in lead V3 [SV3] and R wave in lead V3 [RV3]+SV3). Robustness analysis for sensitivity showed RV3+SV3 was superior to RI+SV3. For all patients after diagnosis, RI+SV4 was the main candidate. However, conventional criteria were more useful than voltage criteria. CONCLUSIONS: Early HCM prediction was possible using RV3+SV3 in >50% of patients in 1st grade. Voltage criteria may help diagnose prediagnostic or early HCM, and prevent tragic accidents, although further prospective studies are required.

Transvenous lead performance of implantable cardioverter-defibrillators and pacemakers.

BACKGROUND: After the reports of recalled leads, several technological improvements have been introduced and the durability of implantable cardioverter defibrillator (ICD) leads has improved. The incidence of lead failures is now less than in the previous studies. However, there are few reports that have shown the long-term durability of ICD leads as compared to pacemaker (PM) leads. This study analyzed the medium to long-term performance of transvenous ICD leads as compared to PM leads. METHODS: We retrospectively studied 1227 cases from April 2007 to December 2017 who underwent an initial transvenous ICD or PM implantation. The number of lead failures and patient background characteristics were analyzed. RESULTS: During a median 3-3.5 years follow up period, 1 (0.3%) ICD lead and 18 (2.4%) PM leads failed. The incidence of lead failures was significantly higher in the PM group than ICD group (p = .019). Males were associated with a higher incidence of lead failures in the PM group. CONCLUSION: Since the era of recalled ICD leads, the durability of ICD leads has remarkably improved and the incidence of lead failures with non-recalled ICD leads has been less than that for PM leads.

Efficacy and safety of tolvaptan after pediatric congenital heart disease surgery.

It is not clear whether tolvaptan is safe and effective irrespective of various underlying clinical conditions including the functional ventricle morphology, chromosomal abnormalities, and renal function after complex pediatric congenital heart disease surgery. Also, the appropriate dose of tolvaptan in these patients has not been previously identified. We retrospectively assessed the urine volume, body weight, patient clinical characteristics, laboratory data, and vital signs before and on days 1 and 7 of the tolvaptan administration after congenital heart disease surgery. Also, we assessed the relationship between the tolvaptan dose and its effects. A total of 86 patients were included the study. The mean time from the surgery to the tolvaptan administration was 23.5 ± 3.7 days. After administering tolvaptan, the urine volume significantly increased and body weight significantly decreased from baseline by days 1 and 7 (p < 0.0001). The urine volume significantly increased more in the survivors than the deceased. Of the 22 patients who had low serum sodium concentrations at baseline, 20 had an increased serum sodium concentration on day 7. The clinical effect of tolvaptan was not affected by the functional ventricle morphology, chromosomal abnormalities, or renal function. There was a positive correlation between the tolvaptan dose and change in the urine volume until a tolvaptan dose of up to 0.3 mg/kg/day but not at more than 0.3 mg/kg/day. Tolvaptan administration is safe and effective after congenital heart disease surgery irrespective of various underlying clinical conditions. Though the urine volume tends to increase until a tolvaptan dose of up to 0.3 mg/kg/day in pediatric congenital heart disease patients, there was no further benefit with more than 0.3 mg/kg/day.

Late recovery of the cardiopulmonary exercise capacity after transcatheter amplatzer device closures for atrial septal defects in adults.

Transcatheter atrial septal defect (ASD) closures using an Amplatzer Septal Occluder (ASO) have been widely performed. Compared to children, we sometimes experience late recovery of exercise performance in adult patients. Our study aimed to evaluate the change in the cardiopulmonary exercise capacity in asymptomatic or mildly symptomatic adult patients after a transcatheter ASD closure using an ASO. The subjects consisted of 29 patients (age 39.5 ± 13.6 years) that underwent cardiopulmonary exercise testing (CPX) before, 3, 6, and 12 months after a transcatheter secundum ASD closure using an ASO. The peak oxygen consumption (peak VO2), anaerobic threshold (AT), and slope of the correlation between the ventilation and carbon dioxide production (VE/VCO2 slope) were evaluated. We also evaluated the left-ventricular end-diastolic diameter (LVEDD), right-ventricular end-diastolic dimension (RVEDD) by echocardiography, and hemodynamic values by cardiac catheterization before the ASO procedure. The peak VO2 did not show any improvement 3 months after the ASO procedure; however, a significant improvement was displayed 6 and 12 months (baseline: 23.4 ± 6.3, 3 months: 23.6 ± 6.4, 6 months: 25.1 ± 5.6, 12 months: 26.4 ± 5.3 mL/kg/min; p < 0.001) after the ASO. The LVEDD (before: 38.1 ± 3.6, 3 months: 43.4 ± 3.4 mm; p < 0.001) and RVEDD (before: 33.6 ± 5.3, 3 months: 26.3 ± 2.6 mm; p < 0.001) on echocardiography quickly improved 3 months after the ASO. Although the LVEDD and RVEDD normalized 3 months after the ASO, the peak VO2 still decreased; however, the peak VO2 improved to almost a normal range 6 months after the ASO.

Diagnostic value of P-waves in children with idiopathic restrictive cardiomyopathy.

Restrictive cardiomyopathy (RCM) is a rare myocardial disease with an impaired diastolic function and poor prognosis. Almost all RCM patients are reported to have abnormal P-waves due to atrial overloading. This study aimed to reveal the characteristics of the P-waves in RCM patients and to suggest the diagnostic index of RCM in children with a 12-lead electrocardiogram (ECG). We retrospectively investigated 17 ECGs of children with idiopathic RCM during the initial visit at 15 institutes in Japan between 1979 and 2013. The RCM group was divided into four groups based on the age (elementary school [ES] and junior high school [JHS] students) and inception of the diagnosis (abnormal ECG on school-heart-screening [e-RCM] and some cardiovascular symptoms [s-RCM]), the ES/e-RCM (n = 5), ES/s-RCM (n = 4), JHS/e-RCM (n = 4), and JHS/s-RCM (n = 4) groups. As an aged-match control group, school-heart-screening ECGs of 1st-grade ES students (16,770 students) and 1st-grade JHS students (18,126 students) from Kagoshima in 2016 were adopted. For a comparison between the groups, we used the effect size "Hedge's g" by calculating the mean and standard deviation of the two groups. An effect size of 0.8 (or above) had an overlap of 53% (or less). The effect sizes of the sum of the absolute values of the forward and backward amplitudes in lead V1 (P1 + P2 V1) was the largest, and the ES/e-RCM, ES/s-RCM, JHS/e-RCM, and JHS/s-RCM were 15.8, 22.1, 9.4, and 10.3, respectively. A P1 + P2 V1 > 200 µV was able to rule in all RCM patients, thus, we proposed 200 µV as the cutoff value for screening purposes. In conclusion, the P1 + P2 V1 in the school-heart-screening may be useful for detecting asymptomatic or early-stage RCM in school-age children.

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome.

An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD). This report describes the efficacy of S-ICDs in a child with an ER pattern after aborted sudden cardiac death.

The Impact of the Pulmonary Artery Index and Aortopulmonary Collateral Artery Coil Embolization on Intractable Pleural Effusions After a Fontan Surgery.

An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization against a pleural effusion before a Fontan operation is still controversial.This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first. Factors associated with the duration of pleural drainage (median: 6 (2-41) days) and that of postoperative hospital stay (median: 25 (14-91) days) were analyzed using a multiple regression analysis. The pulmonary artery index (PAI; Nakata index) was associated with both the pleural drainage duration (P < 0.05, r2 = 0.17) and postoperative hospital stay (P < 0.05, r2 = 0.10).Thereafter, all the 227 patients were classified into the following three groups: Group A (12 patients in whom the embolization was performed within 30 days before the Fontan surgery), Group B (131 patients in whom the embolization was performed more than 30 days before the Fontan surgery), and Group C (84 patients in whom the embolization was not performed). Patients in Group A were found to be associated with the shortest length of both periods (P < 0.05).Lower PAI values were related to a prolonged pleural drainage duration and postoperative hospital stay. APC coil embolizations may reduce the risk if they are performed shortly (less than 30 days) before the operation.

Successful Percutaneous Thrombectomy of the Left Pulmonary Artery in a Neonate After a Patent Ductus Arteriosus Clipping.

We report a neonate with a successful percutaneous thrombectomy of a total thrombotic occlusion of the left pulmonary artery (LPA) after a surgical clipping for a patent ductus arteriosus (PDA). We suspected the compression of the LPA by the clipping and postoperative hemodynamic instability caused the LPA obstruction. After the surgical removal of the PDA clip and division of the PDA, we could safely retrieve the LPA thrombus with a non-hydrodynamic thrombectomy catheter for coronary arteries.

Early assessment of cardiac troponin I predicts the postoperative cardiac status and clinical course after congenital heart disease surgery.

Cardiac troponin I (cTnI) is a regulatory protein with a high sensitivity and specificity for cardiac injury. Preoperative and postoperative elevations of cTnI are usually considered predictors of the mortality and morbidity. However, little is known about the relationship between the cTnI and postoperative course after the congenital heart disease (CHD) operation. Sixty-five consecutive patients who underwent cardiac surgery for CHD at our institution between March 2016 and January 2017 were included. The cTnI was measured after the operation. Also, the association between the cTnI and duration of the catecholamine use, ICU stay, aortic cross clamp time, and other clinical parameters were assessed. The cTnI level on postoperative day 1 was positively correlated with the duration of the catecholamine use (p < 0.001) and ICU stay (p < 0.001). Also, a higher cTnI level was associated with a lower urine volume and higher lactate level 24 h after the ICU admission. In the multivariable regression analysis, the cTnI was a significant independent predictor of the catecholamine use (p = 0.002) and ICU stay (p = 0.003). The cTnI level on postoperative day 1 was a predictor of the duration of the catecholamine use and ICU stay.