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The left ventricular (LV) apex is recommended as the first choice for positioning the epicardial pacing. We encountered a patient with congenital heart disease (CHD) showing hypokinesis of the LV apical pacing site after implantation of a pacemaker with epicardial leads. This phenomenon was revealed by the early shortening and systolic rebound stretch of the same lesion on two-dimensional speckle tracking echocardiography, which developed in the intraventricular dyssynchrony between the LV apex and base. Cardiac resynchronization therapy provided an excellent result around the hypokinetic lesion. It is wise to arrange detailed evaluations in each patient with complicated CHD, aiming at a successful treatment to enable ventricular synchronicity.

Catheter intervention (CI) for a Blalock-Taussig shunt (BTS) or a ventricle-to-pulmonary artery conduit (VPC) is often required after a palliative surgery for congenital heart disease. Flow regulatory clips help improve interstage mortality; their use necessitates CIs to prevent cyanosis. To study the CI outcomes in patients who underwent palliative surgery with either BTSs or VPCs with flow regulatory clips. This single-center retrospective study evaluated demographic characteristics and interventional outcomes of 49 consecutive pediatric patients who required CI for BTS (BTS group) or VPC (VPC group) between January 2008 and September 2018. Overall, 34 and 18 procedures were performed in the BTS and VPC groups, respectively. Moreover, 19/32 (59.3%) and 12/17 (70.1%) patients from the BTS and VPC groups had flow regulatory clips, respectively. All clips were unclipped successfully; one patient in each group underwent staged unclipping. A higher proportion of "clipped patients" underwent CI due to desaturation [clipped vs. non-clipped: BTS, 10/20 (50.0%) vs. 3/14 (21.4%), p = 0.092; VPC, 9/13 (69.2%) vs. 1/5 (20.0%), p = 0.060]. Most clipped patients successfully progressed to the next stage [BTS, 19/20 (95.0%); VPC, 12/13 (92.3%)]. Severe adverse events (SAEs) were more frequent in the VPC group than in the BTS group [3/13 (23.1%) vs. 0/20 (0%), p = 0.024]. Two patients developed an atrioventricular block (requiring an atropine infusion), while one died due to pulmonary overcirculation. While the indication of CI was cyanosis for a higher proportion of clipped patients, all clips were unclipped successfully. The incidence of CI-related SAEs was higher in the VPC group than in the BTS group.

ObjectiveFontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening and management are lacking. The present study aimed to identify the predictors of liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in post-Fontan patients.MethodsThis was a multi-institutional retrospective cohort study. Clinical data of all perioperative survivors of Fontan operation before 2011 who underwent postoperative catheterisation were collected through a retrospective chart review.ResultsA total of 1117 patients (538 women, 48.2%) underwent their first Fontan operation at a median age of 3.4 years. Postoperative cardiac catheterisation was conducted at a median of 1.0 year. During a median follow-up period of 10.3 years, 67 patients (6.0%) died; 181 (16.2%) were diagnosed with liver fibrosis, 67 (6.0%) with LC, 54 (4.8%) with focal nodular hyperplasia and 7 (0.6%) with HCC. On multivariable analysis, high central venous pressure (CVP) (HR, 1.28 (95% CI 1.01 to 1.63) per 3 mm Hg; p=0.042) and severe atrioventricular valve regurgitation (HR, 6.02 (95% CI 1.53 to 23.77); p=0.010) at the postoperative catheterisation were identified as independent predictors of LC/HCC.ConclusionsPatients with high CVP and/or severe atrioventricular valve regurgitation approximately 1 year after the Fontan operation are at increased risk of developing advanced liver disease in the long term. Whether therapeutic interventions to reduce CVP and atrioventricular valve regurgitation decrease the incidence of advanced liver disease requires further elucidation.

The patient was a 28-year-old woman who was misdiagnosed with tetralogy of Fallot and straddling mitral valve after birth. She underwent a left modified Blalock-Taussig shunt at the age of 1 year. At age 28, she presented with fatigue and progressive cyanosis. Finally, she was diagnosed with tetralogy of Fallot and complete atrioventricular septal defect. To measure the exact biventricular volumes, we performed cardiac magnetic resonance imaging in addition to cardiac angiography and ensured adequate volume capacity. We eventually decided to perform biventricular repair. Her postoperative course was uneventful, and she returned to full-time work.

Transcatheter closure is an established method to treat coronary artery fistula (CAF). We present transcatheter closure in a 6-year-old girl with CAF and anomalous aortic origin of the left main coronary artery from the right aortic sinus. The CAF originated from the left coronary artery (LCA), coursed through the interventricular septum (intraseptal course) with prominent dilation, and drained into the right ventricular outflow tract. She underwent transcatheter closure and was in a stable condition at the 3-year follow-up with regression of the dilated portion of the intraseptal-type LCA. Hence, transcatheter closure of CAF is feasible in patients with anomalous origins of coronary arteries.

Background Congenital porto-systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy. One technique came from evaluating patients with extrahepatic portal vein obstruction (EHPVO). Methods We conducted a single center retrospective study to evaluate the efficacy of angiographic diagnostic procedure for the assessment of CPSS and EHPVO, and its impact on patients' subsequent interventions and clinical course. Eight patients with CPSS and two patients with EHPVO who underwent diagnostic angiography were included. Assessment of intrahepatic portal vein was performed in all patients. The route of the shunt, and portal vein pressure under shunt occlusion were also evaluated for patients with CPSS. Evaluation was first attempted with balloon angiographic catheter (standard method). Three additional techniques, 1) direct wedge-catheter injection without balloon inflation, 2) use of occlusion balloon in two patients, and 3) hybrid angiography with sheath placement directly into the superior mesenteric vein were performed as needed. Results The standard method was sufficient in four patients. Contrarily, all three techniques were required in two patients each. One lost contact during follow up, but all other patients underwent optimal intervention. There was no complication related to the angiographic procedure. Conclusions Use of direct wedge-catheter injection without balloon inflation, occlusion balloon, and hybrid catheterization improved the diagnostic yield in patients with CPSS or EHPVO.

Background Standardized treatment of fetal tachyarrhythmia has not been established. Objectives This study sought to evaluate the safety and efficacy of protocol-defined transplacental treatment for fetal supraventricular tachycardia (SVT) and atrial flutter (AFL). Methods In this multicenter, single-arm trial, protocol-defined transplacental treatment using digoxin, sotalol, and flecainide was performed for singleton pregnancies from 22 to Results A total of 50 patients were enrolled at 15 institutions in Japan from 2010 to 2017; short ventriculoatrial (VA) SVT (n = 17), long VA SVT (n = 4), and AFL (n = 29). One patient with AFL was excluded because of withdrawal of consent. Fetal tachyarrhythmia resolved in 89.8% (44 of 49) of cases overall and in 75.0% (3 of 4) of cases of fetal hydrops. Pre-term births occurred in 20.4% (10 of 49) of patients. Maternal AEs were observed in 78.0% (39 of 50) of patients. Serious AEs occurred in 1 mother and 4 fetuses, thus resulting in discontinuation of protocol treatment in 4 patients. Two fetal deaths occurred, mainly caused by heart failure. Neonatal tachyarrhythmia was observed in 31.9% (15 of 47) of neonates within 2 weeks after birth. Conclusions Protocol-defined transplacental treatment for fetal SVT and AFL was effective and tolerable in 90% of patients. However, it should be kept in mind that serious AEs may take place in fetuses and that tachyarrhythmias may recur within the first 2 weeks after birth.

An extremely low birth weight infant (810 g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic findings, we performed banding operations twice to regulate the pulmonary blood flow on day 2 and day 9. At 6 months of age, a definitive repair simultaneous with unifocalization of major aortopulmonary collateral arteries was performed. At 1 year of age, the right ventricle/left ventricle pressure ratio was 0.44 after balloon angioplasty was performed for the right-sided pulmonary artery stenosis. The patient is in a stable condition and was followed-up for more than 2 years after definitive repair. This is the first known successful repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated. A total of 180 patients were identified. The median age at diagnosis was 5.8 years old and median duration of observation was 8.3 years. Although six patients (3%) deteriorated into the dilated phase of HCM, no patient received heart transplantation. Freedom from death at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively. There were 26 deaths. Among them, 11 patients died suddenly, presumably due to arrhythmia, and 15 patients died of heart failure. The presence of heart failure symptoms and a greater cardiothoracic ratio were significant risk factors for heart failure-related death. There were no significant risk factors identified for arrhythmia-related death. In conclusion, the prognosis of pediatric HCM in Japan is good and similar to those reported in population-based studies in the United States and Australia. Significant risk factors for heart failure-related death were identified in pediatric patients with HCM in Japan.

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 Left ventricular function has been shown to be an important prognostic indicator in patients with repaired tetralogy of Fallot (rTOF) and tends to decrease with age. In recent years, left atrial (LA) function was reported to be a useful prognostic indicator more than or equal to left ventricular function in acquired heart diseases. However, atrial function in rTOF has not yet been sufficiently examined. PURPOSE The aim of this study was to investigate the relationship between LA dysfunction and age in rTOF using strain analysis. METHODS In order to assess the relationship between LA function and age, we recruited 59 patients with rTOF ranging in age from 5-40 years. We stratified the patients into 3 groups (T1: 5-10 years, T2: 11-20 years, T3: 21-40 years) and divided 54 controls of similar age into 3 corresponding groups (N1, N2, and N3). Two-dimensional speckle tracking images (2D-STI) obtained from four- and two-chamber views were used to assess LA functions by measuring reservoir, conduit, and pump strain. Additionally, we measured the strain rate (SR) in the systole, early diastole, and late diastole. RESULTS LA reservoir strain (37.4 ± 2.2% vs. 47.9 ± 1.7%, P= 0.004), LA pump strain (8.3 ± 1.4% vs. 14.1 ± 2.7%, p Early diastole SR was significantly decreased in the T1 and T3 groups compared with the N1 and N3 groups (T1 vs N1, 3.00 ± 0.63% vs. 4.03 ± 0.0.80%, p CONCLUSIONS In rTOF, most of the LA functions assessed by strain analysis decreased compared to normal controls in over 20 years old age. Furthermore, all measured functions decreased with age in rTOF while only two parameters decreased with age in normal controls. These results suggest that LA function may be an important indicator in long-term rTOF follow-up. These are new insights into LA function in patients with rTOF.

Background Anthracycline cardiotoxicity affects clinical outcomes, and its early detection using methods that rely on conventional echocardiography, such as left ventricular ejection fraction (LVEF) is difficult. This study aimed to evaluate the characteristics and the differences in cardiac dysfunction among childhood cancer survivors in 3 age groups using layer-specific strain analysis in a wide age range.Methods and Results:The 56 patients (median age: 15 [range: 6.8-40.2] years) who had been treated with anthracycline for childhood cancer were divided into 3 age groups (C1: 6-12 years, C2: 13-19 years, C3: 20-40 years) after anthracycline treatment, and 72 controls of similar ages were divided into 3 corresponding groups (N1, N2, and N3). Layer-specific longitudinal strain (LS) and circumferential strain (CS) of 3 myocardial layers (endocardium, midmyocardium, and epicardium) were determined using echocardiography. Myocardial damage had not occurred yet in C1. Endocardial CS at the basal level was less in C2 than in N2. Endocardial CS at all levels and midmyocardial CS at the basal and papillary levels were lower in C3 than in N3. LVEF and LS were not significantly different between patients and controls. Conclusions Among survivors of childhood cancer, impaired myocardial deformation starts in adolescence and extends from the endocardium towards the epicardium and from the base towards the apex with age. These findings are a novel insight into the time course of anthracycline cardiotoxicity.

To investigate the clinical course of fetal tachycardia and analyze the impact of intrauterine treatment on the postnatal treatment and patient outcomes.This was a retrospective review of cases of fetal tachycardia that occurred from 2004 to 2006. Data were collected from questionnaires that were sent to all 750 secondary or tertiary perinatal care centers in Japan.Eighty-two cases (14 with fetal hydrops) were analyzed (supraventricular tachycardia [SVT], n = 52; atrial flutter [AFL], n = 23; and ventricular tachycardia, n = 7). The overall mortality was 3.7%. Intrauterine treatment was performed for 41 fetuses (50.0%). Digoxin, flecainide and sotalol were mainly used for SVT and AFL. Fetal tachycardia resolved in 90.0% (27/30) of the cases without fetal hydrops and 90.9% (10/11) of the cases with fetal hydrops. Intrauterine treatment significantly reduced the incidence of cesarean delivery (29.3 vs. 70.7%, p .01), preterm birth (12.2 vs. 41.5%, p = .02) and neonatal arrhythmias (48.8 vs. 78.0%, p = .01) in comparison to untreated fetuses.This nationwide survey revealed that intrauterine treatment was performed for approximately half of the cases of fetal tachycardia and was associated with lower rates of cesarean delivery, premature birth and neonatal arrhythmias in comparison to untreated fetuses.

Assessment of left ventricular (LV) dysfunction is vital in patients with repaired tetralogy of Fallot (rTOF). The early diastolic intraventricular pressure gradient (IVPG) in the LV plays an important role in diastolic function. IVPG is calculated as the intraventricular pressure difference divided by the LV length, which allows to account for differences in LV size and therefore calculate IVPG in children. We aimed to investigate the mechanisms of LV diastolic dysfunction by measuring mid-to-apical IVPG as an indicator of the active suction force sucking blood from the left atrium into the LV. We included 38 rTOF patients and 101 healthy controls. The study population was stratified based on age group into children (4–9 years), adolescents (10–15 years), and adults (16–40 years). IVPGs were calculated based on mitral inflow measurements obtained using color M-mode Doppler echocardiography. Although total IVPGs did not differ between rTOF patients and controls, mid-to-apical IVPGs in adolescents and adults were smaller among rTOF patients than among controls (0.15 ± 0.05 vs. 0.21 ± 0.06 mmHg/cm, p

Background Although the subclinical left ventricular (LV) dysfunction caused by diabetes mellitus (DM) results in a high risk of death and heart failure, the details of cardiac dysfunction across a wide age range remain unclear. The aim of this study was to assess LV dysfunction in patients with type 1 DM (T1DM) using layer-specific strain analysis by echocardiography.Methods and Results:The 52 patients (median age: 23 [range: 5-40] years) with T1DM were divided into 3 age groups (D1: 5-14 years, D2: 15-24 years, D3: 25-40 years); 78 age- and sex-similar controls were divided into 3 corresponding groups (C1, C2, and C3). Layer-specific longitudinal strain (LS) and circumferential strain (CS) of the 3 myocardial layers (endocardium, midmyocardium, and epicardium) were determined using echocardiography. Strains did not decrease in D1. Epicardial and midmyocardial CS at the basal level and LS in all layers were decreased in D2 compared with C2. CS at the basal level and LS in all layers were lower in D3 than in C3. The strains correlated with the duration of T1DM and LV wall thickness. Conclusions In patients with T1DM, longitudinal deformation in all layers and epicardial and midmyocardial circumferential deformation at the basal level decreased from the late teens, which correlated with the duration of the disease and LV hypertrophy.

Aims Anomalous origin of the coronary artery (AOCA) with an inter-arterial course (IAC) between the great vessels poses a risk for a life-threatening cardiovascular event. We assessed, in a registry-based study, the clinical features, treatment strategies, and prognoses of life-threatening cardiovascular events ensuant to AOCA. Methods and results Included were 65 AOCA patients (48 men/17 women, aged 41 ± 23 years) from 40 clinical centres who had experienced sudden cardiac arrest (SCA) (n = 30), acute myocardial infarction (AMI) (n = 5), angina (n = 23), or syncope (n = 7). The anomalous vessel was the right coronary artery in 72% of patients and left coronary artery in 28%; the ostium was slit-like in 42%. Coronary luminal narrowing ≥75% was absent in patients with SCA or syncope (86% and 57%, respectively), but occlusion or narrowing was seen in those with AMI (100%) or angina (52%). Age ≤40 years, male sex, sporting activity, absence of prodromal symptoms, acutely angled (≤30°) take-off from the aorta, and absence of luminal narrowing of the IAC segment were associated with SCA in this patient group. Coronary vasospasm was inducible in 12 of 17 patients without coronary narrowing. Management included surgical revascularization (n = 26) percutaneous coronary intervention (n = 9), and medical treatment (n = 26). Four SCA patients died while hospitalized; no others died during the median 5.0 (range, 1.8–7.0)-year follow-up period. Conclusions In patients with AOCA, age ≤40 years, male sex, sporting activity, and an acute take-off angle appear to be risk factors for SCA. Appropriate management can be beneficial. Confirmation in a large-scale study is warranted.

Standardized treatment of fetal tachyarrhythmia has not been established.This study sought to evaluate the safety and efficacy of protocol-defined transplacental treatment for fetal supraventricular tachycardia (SVT) and atrial flutter (AFL).In this multicenter, single-arm trial, protocol-defined transplacental treatment using digoxin, sotalol, and flecainide was performed for singleton pregnancies from 22 to 37 weeks of gestation with sustained fetal SVT or AFL ≥180 beats/min. The primary endpoint was resolution of fetal tachyarrhythmia. Secondary endpoints were fetal death, pre-term birth, and neonatal arrhythmia. Adverse events (AEs) were also assessed.A total of 50 patients were enrolled at 15 institutions in Japan from 2010 to 2017; short ventriculoatrial (VA) SVT (n = 17), long VA SVT (n = 4), and AFL (n = 29). One patient with AFL was excluded because of withdrawal of consent. Fetal tachyarrhythmia resolved in 89.8% (44 of 49) of cases overall and in 75.0% (3 of 4) of cases of fetal hydrops. Pre-term births occurred in 20.4% (10 of 49) of patients. Maternal AEs were observed in 78.0% (39 of 50) of patients. Serious AEs occurred in 1 mother and 4 fetuses, thus resulting in discontinuation of protocol treatment in 4 patients. Two fetal deaths occurred, mainly caused by heart failure. Neonatal tachyarrhythmia was observed in 31.9% (15 of 47) of neonates within 2 weeks after birth.Protocol-defined transplacental treatment for fetal SVT and AFL was effective and tolerable in 90% of patients. However, it should be kept in mind that serious AEs may take place in fetuses and that tachyarrhythmias may recur within the first 2 weeks after birth.

Although mutations in several genes have been reported in pulmonary arterial hypertension (PAH), most of PAH cases do not carry these mutations. This study aimed to identify a novel cause of PAH. To determine the disease-causing variants, direct sequencing and multiplex ligation-dependent probe amplification were performed to analyze 18 families with multiple affected family members with PAH. In one of the 18 families with PAH, no disease-causing variants were found in any of BMPR2, ACVRL1, ENG, SMAD1/4/8, BMPR1B, NOTCH3, CAV1, or KCNK3. In this family, a female proband and her paternal aunt developed PAH in their childhood. Whole-exome next-generation sequencing was performed in the 2 PAH patients and the proband’s healthy mother, and a BRCA1-associated protein (BRAP) gene variant, p.Arg554Leu, was identified in the 2 family members with PAH, but not in the proband’s mother without PAH. Functional analyses were performed using human pulmonary arterial smooth muscle cells (hPASMCs). Knockdown of BRAP via small interfering RNA in hPASMCs induced p53 signaling pathway activation and decreased cell proliferation. Overexpression of either wild-type BRAP or p.Arg554Leu-BRAP cDNA constructs caused cell death confounding these studies, however we observed higher levels of p53 signaling inactivation and hPASMC proliferation in cells expressing p.Arg554Leu-BRAP compared to wild-type BRAP. In addition, p.Arg554Leu-BRAP induced decreased apoptosis of hPASMCs compared with wild-type BRAP. In conclusion, we have identified a novel variant of BRAP in a Japanese family with PAH and our results suggest it could have a gain-of-function. This study sheds light on new mechanism of PAH pathogenesis.

Background The purpose of this study was to investigate fetal cardiac ultrasonographic parameters in relation to the outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH). Methods Subjects comprised 33 fetuses with fetal CDH and 99 control fetuses without cardiac and lung anomalies. Three parameters, cardiothoracic area ratio (CTAR), the ratio of the diameter of the main pulmonary artery to that of the ascending aorta (MPA/AAo), and the ratio of the diameter of the tricuspid valve to that of the mitral valve (TV/MV) were compared between fetal CDH and control subjects and between survival (SG) and non-survival (NSG) groups. Results All three parameters differed significantly between CDH and control fetuses. Significant differences between SG and NSG were observed in CTAR and TV/MV ratio, but not in MPA/AAo ratio before and after 32 gestational weeks. A significant increase in the TV/MV ratio was noted along with an increase in gestational age. A TV/MV ratio of >1.72 discriminated non-survivors from survivors with better sensitivity and specificity. Conclusions Fetal cardiac ultrasonography provides useful information for predicting the outcomes of isolated left-sided CDH. The TV/MV ratio was considered a reliable indicator that reflected outcomes of isolated left-sided CDH.

Cardiac dysfunction due to cardiotoxicity from anthracycline chemotherapy is a leading cause of morbidity and mortality in survivors of childhood cancer. The intraventricular pressure gradient (IVPG) of the left ventricle (LV) is the suction force of blood from the left atrium to the LV apex during early diastole and is a sensitive indicator of diastolic function. We assessed IVPG as a new indicator of the cardiac dysfunction in survivors of childhood cancer after anthracycline therapy. We performed a prospective echocardiographic study on 40 survivors of childhood cancer aged 6–26 years who received anthracycline therapy (group A) and 53 similar-age normal controls (group N). The subjects were divided into the younger groups, N1 and A1 (age < 16 years); older groups, N2 and A2 (age ≥ 16 years). IVPG was calculated using color M-mode Doppler imaging of the mitral inflow using Euler’s equation. Total IVPG was divided into the basal and mid-to-apical IVPG to demonstrate more clearly the mechanisms of the LV diastolic suction force. The total anthracycline dose was 16.2–600.0 mg/m2 (median 143.5 mg/m2). Total IVPG significantly decreased in group A2 compared with that in group N2 (0.39 ± 0.07 vs. 0.29 ± 0.11 mmHg/cm; p = 0.010). The mid-to-apical IVPG significantly decreased in groups A1 and A2 compared with that in groups N1 and N2, respectively (N1 vs. A1: 0.20 ± 0.05 vs. 0.16 ± 0.05 mmHg/cm, p = 0.036; N2 vs. A2: 0.21 ± 0.06 vs. 0.14 ± 0.06 mmHg/cm, p = 0.001). Basal IVPG, E wave, and E/e′ were not significantly different between patients and normal controls. The total and mid-to-apical IVPG, especially mid-to-apical IVPG, could be sensitive new indicators in survivors of childhood cancer after anthracycline therapy.

Background: Standardized treatment of fetal supraventricular tachycardia (SVT) and atrial flutter (AFL) has not been established. The aim of this study was to evaluate the safety and efficacy of protocol-defined transplacental treatment for fetal SVT and AFL. Methods: In this multicentre single-arm trial (UMIN Clinical Trials Registry number 000004270), protocol-defined transplacental treatment using digoxin, sotalol, and flecainide was performed for singletons from 22 to

Although the suction force that moves blood into the left ventricle during early diastole is thought to play an important role in diastolic function, there have been a few studies of this phenomenon in normal children. Suction force is measured as the intraventricular pressure difference (IVPD) and intraventricular pressure gradient (IVPG), which is calculated as IVPD divided by left ventricular length. The purpose of this study was to determine the suction force in infants, children, and adolescents using IVPD and IVPG. We included 120 normal children categorized into five groups based on age: G1 (0–2 years), G2 (3–5 years), G3 (6–8 years), G4 (9–11 years), and G5 (12–16 years). The total, basal, and mid–apical IVPD and IVPG were calculated using color M-mode Doppler imaging of the mitral valve inflow using the Euler equation. The total IVPD increased with age from G1 to G5 (1.75 + 0.51 vs. 2.95 + 0.72 mmHg, respectively; p

BACKGROUND Because there is limited information on fetal bradyarrhythmia associated with congenital heart defects (CHD), we investigated its prognosis and risk factors. METHODS AND RESULTS In our previous nationwide survey of fetal bradyarrhythmia from 2002 to 2008, 38 fetuses had associated CHD. Detailed clinical data were collected from secondary questionnaires on 29 fetuses from 18 institutions, and were analyzed. The 29 fetuses included 22 with isomerism, 4 with corrected transposition of the great arteries (TGA) and 3 with critical pulmonary stenosis; 14 had complete atrioventricular block (AVB), 8 had second-degree AVB, and 16 had sick sinus syndrome; 5 died before birth, and 10 died after birth (5 in the neonatal period). Neonatal and overall survival rates for fetal bradyarrhythmia with CHD were 66% and 48%, respectively. Pacemaker implantation was needed in 17 cases (89%). Beta-sympathomimetics were administered in utero in 13 cases and were effective in 6, but were not associated with prognosis. All cases of corrected TGA or ventricular rate ≥70 beats/min survived. A ventricular rate

Aims Real time 3D echocardiography (RT3DE) has been applied for the assessment of left atrial (LA) function in patients with adult heart disease; however, LA function is not well known in children. We aimed at determining the normal range of LA volume (LAV) using RT3DE and the feasibility and reproducibility of this method in healthy subjects and at elucidating the developmental changes in the LAV with aging. Methods and Results In this study, 359 healthy people (mean age, 23.9 ± 21.3; range, 0.1–76.4 years) were enrolled. We performed transthoracic RT3DE and measured the maximum and minimum LAV. Simultaneously, we measured the LAV using the 2D biplane Simpson's method. Inter-observer and intra-observer variability and the agreement of LAV measurements between RT3DE and 2DE were assessed in a subset of subjects. The RT3DE feasibility for LAV measurement was 93%. Both maximum and minimum LAVs exponentially increased with age and linearly increased with increasing of body surface area (BSA). The LA distensibility, which demonstrates LA reservoir function, decreased with age and BSA. The LAVs measured by RT3DE were significantly smaller than those measured by the 2D biplane Simpson's method. The 3D volumetric method had favorable intra-observer and inter-observer agreement. Conclusion The reference values of LAV from early infancy to adulthood were obtained using RT3DE, which could be useful for future studies in children with congenital heart disease. RT3DE is a reproducible method and a feasible tool for evaluating the LAV in children. LA reservoir function is likely to decrease with age and increasing of body size.

Background: Circumstances and outcomes of out-of-hospital cardiac arrest (OHCA) in elementary and middle school students while at school in the era of public-access defibrillation are unknown. Methods and Results: We conducted a nationwide hospital-based survey of elementary and middle school students who had had OHCA of cardiac origin and received prehospital resuscitation in 2005–2009. Among 58 cases recruited, 90% were witnessed by bystanders; 86% had ventricular fibrillation as the initial rhythm; 74% were resuscitated by bystanders; 24% were defibrillated by bystanders; 55% occurred at school; 66% were exercise-related; 48% were followed up before the event; 67% had structural heart disease. In total, 53% of overall patients and 79% of those initially defibrillated by bystanders had a favorable neurological outcome. Patients were more likely to be defibrillated by bystanders (38% vs. 8%, P=0.012) and had a more favorable neurological outcome in schools (69% vs. 35%, P=0.017) than in other locations. The majority of arrests in schools were exercise-related (84% vs. 42%, P=0.001), occurred at sports venues, and students were resuscitated by teachers; half of the cases at school occurred in patients with a pre-event follow-up. Conclusions: After OHCA, children were more likely to be defibrillated by bystanders and had a better outcome in schools than in other locations, which may be relevant to the circumstances of events. (Circ J 2014; 78: 701–707)

Background: Transcatheter atrial septal defect occlusion (ASO) results in increased end-diastolic and stroke volume of left ventricle (LV), thereby improving exercise capacity in the chronic phase. However, not all patients demonstrate LV volume dilation after ASO. The aim of this study is to assess the change of LV volume after ASO and the relationship between the diastolic LV function and the grade of LV volume expansion after ASO. Methods: Thirty-five patients (median, 19.2 years; range, 6.9–67.5) who underwent successful ASO and sequential LV volume analyses using real-time three-dimensional echocardiography were included in this study. LV volume and LV diastolic function were assessed at baseline, at 48 hours, and at 3 months after ASO. Serum concentrations of natriuretic peptides were measured simultaneously. Results: LV end-diastolic volume index was 40.8 (25.6–62.9) ml/m2 at baseline, 45.5 (26.1–72.5) (p