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This study investigated the clinical outcomes and the effect of band tightness on outcome after pulmonary artery banding (PAB) in patients with complete atrioventricular septal defect (AVSD).From 2000 through 2019, among 133 patients with isolated complete AVSD pursuing biventricular repair, 34 patients (25.6%) who underwent PAB were included in this study. Factors associated with adverse outcome, which was defined as prolonged stay in the intensive care unit (10 days), were analyzed using a multiple logistic regression model. Receiver operating characteristic analysis was performed to identify a threshold band tightness for adverse outcome.The median age and weight were 43 days and 3.6 kg, respectively. There were 4 early deaths. The median intensive care unit stay was 8 days. Twenty-eight patients (28/34; 82.4%) underwent definitive repair 10 months (interquartile range, 7-12 months) after PAB. In multivariable analysis, indexed band diameter was identified as a factor associated with adverse outcome (odds ratio, 1.60; 95% CI, 1.03-2.48; P = .035). Receiver operating characteristic analysis indicated 22.2 mm/mPAB is a viable option for early-presenting patients with complete AVSD. Tighter PAB might be beneficial for early postoperative outcomes and preventing progression of atrioventricular valve regurgitation in complete AVSD.

OBJECTIVES This study investigated the effect of a bicuspid pulmonary valve on neoaortic root morphology, function, and the clinical outcomes of early survivors after the arterial switch operation using propensity score matching. METHODS From 1997 to 2018, a total of 442 patients underwent the arterial switch operation for transposition of the great arteries. After exclusion of patients who underwent a staged repair, were repaired beyond 1 year of age, died before discharge and who lacked echocardiographic data at discharge, propensity score matching was used for analysis. RESULTS Among 352 eligible patients, 18 patients (5.1%) had a bicuspid pulmonary valve. After propensity score matching (1:4), 15 patients with bicuspid valve (bicuspid group) and 60 patients with tricuspid valve (tricuspid group) were enrolled. The median follow-up duration was 9.9 years (4 months∼22.3 years). All-cause reoperation-free survival at 10 years was 93.3% in the bicuspid group and 87.0% in the tricuspid group (P = 0.839), and reoperation for neoaortic valve or root was required in 2 patients in the bicuspid group and 1 in the tricuspid group without intergroup difference. The z-score of the neoaortic annulus did not change in either group, although there was an increasing tendency in the z-score of the neoaortic sinus without intergroup difference (P = 0.690). Deterioration in neoaortic valve function was more prominent in the bicuspid group (p = 0.028). CONCLUSIONS The neoaortic sinus might outgrow the norm regardless of the number of neoaortic valve cusps, whereas the neoaortic annulus remained unchanged. Deterioration of valve function was more prominent in the bicuspid group, which suggests that a bicuspid valve might play a significant role in deterioration of neoaortic valve function, without an additional effect on root pathology.

This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty.From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms. Computed tomography was used to measure the narrowest tracheal diameter.The median age and weight at repair were 7.6 months and 7.7 kg, respectively. Most patients (20 out of 21, 95.2%) had preoperative respiratory symptoms. Associated airway anomalies included tracheal ring in 12 (57.1%), bridging bronchus in 8 (38.1%), and tracheal bronchus in 2 patients (9.5%). There was 1 in-hospital death (4.8%). The median ventilator time and intensive care unit stay were 23 hours and 3 days, respectively. There was neither late death nor tracheal intervention during follow-up. Five patients (25.0%) underwent reintervention for left PA stenosis. Hospital readmission for respiratory symptom was required in 7 patients and was associated with the narrowest preoperative tracheal diameter (P = .025) and cardiopulmonary bypass time (P = .040) in univariable analysis. The narrowest tracheal diameter of 3.4 mm was identified as a cutoff value for readmission for respiratory symptom. Freedom from readmission for respiratory symptom was 63.3% at 10 years.PA sling repair without tracheal surgery might be a reasonable surgical option with rare need for tracheal intervention. Hospital readmissions for respiratory symptoms are more frequently required in patients with smaller tracheal diameter and all readmissions were limited to within 2 years after repair.

BACKGROUND Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.Methods and Results:Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019. Altogether, 99 patients were enrolled (mean age 13.9±4.1 years). The most common underlying disease was a primary electrical disease (56%). An ICD was implanted for primary prevention in 19%. Appropriate shock occurred in 44% of patients at a median of 1.6 years after implantation. There was no significant difference in the appropriate shock rate between patients with primary and secondary prevention indications (32% vs. 48%, respectively). A total of 33 patients (33%) experienced inappropriate shock, which was associated with primary electrical disease and follow-up duration on multivariate analysis. 17% of patients had ICD-related complications. CONCLUSIONS The utilization rate of ICD for primary prevention was still low in the pediatric population in Korea, but there was a substantial rate of appropriate shock in these patients. Efforts to increase ICD usage to save the lives of high-risk patients and reduce the incidence of inappropriate shock are required.

Background Pre-Fontan risk stratification is still less than optimal. We aimed to evaluate the effectiveness of intraoperative pulmonary flow study (IOFS), in combination with conventional preoperative evaluation, in the identification of high-risk candidates for the Fontan operation. Method Data from 37 patients (age: 38.1 ± 4.9 months) who underwent the Fontan operation with IOFS were retrospectively reviewed. After anastomosing the Gore-Tex vascular graft to the confluent pulmonary artery, IOFS was performed through the graft at various cardiac indices (CI) (1–4 L/min/m2) generated from a roller pump. Mean pulmonary artery pressure (mPAP) and left atrial pressure (LAP) were recorded. The patients were divided into two groups: those who required Fontan circuit fenestration (group Ff) and those who did not (group Fn). Results Eleven patients (29.7%) required fenestration, intraoperatively (n = 4) and postoperatively (n = 7), due to hemodynamic instability. Heterotaxy syndrome (P = 0.005) and atrioventricular valvar regurgitation (P = 0.04) were more frequent, and ventricle posterior wall thickness (VPWT) was higher (P = 0.024) in group Ff. On IOFS, the increase in mPAP by CI was sharper in group Ff than in group Fn (P Conclusions IOFS may be helpful to identify high-risk Fontan candidates, and high mPAP at CI of 3.0 L/min/m2 on IOFS is a significant predictor of the requirement for fenestration. VPWT is an important preoperative risk factor which correlates with the IOFS results.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1–2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled. We excluded patients diagnosed with cardiac allograft vasculopathy or with an episode of rejection 1 month before or after their echocardiographic examinations. The mean LVGLS was significantly reduced at 1 month when compared to 1 year following transplant (− 15.5% vs. − 19.4%, respectively, p

Background:This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years.Methods:In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan–Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model.Results:The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948–271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047–15.376; p = 0.043).Conclusion:Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.

BACKGROUND: We investigated preoperative cerebral (ScO2) and abdominal (StO2) regional oxygen saturations according to cardiac diagnosis in neonates with critical CHD, their time trends, and the clinical and biochemical parameters associated with them. METHODS: Thirty-seven neonates with a prenatal diagnosis of CHD were included. ScO2 and StO2 values were continuously evaluated using near-infrared spectroscopy. Measurements were obtained hourly before surgery. A linear mixed effects model was used to assess the effects of time and cardiac diagnosis on regional oxygenation and to explore the contributing factors. RESULTS: Regional oxygenation differed according to cardiac diagnosis (p &lt, 0.001). ScO2 was lowest in the patients with severe atrioventricular valvar regurgitation (AVVR) (48.1 ± 8.0%). StO2 tended to be lower than ScO2, and both worsened gradually during the period between birth and surgery. There was also a significant interaction between cardiac diagnosis and time. The factors related to ScO2 were hemoglobin and arterial saturation, whereas no factor was associated with StO2. CONCLUSIONS: Preoperative ScO2 and StO2 in critical CHD differed according to cardiac diagnosis. ScO2 in the patients with severe AVVR was very low, which may imply cerebral hypoxia. ScO2 gradually decreased, suggesting that the longer the time to surgery, the higher the risk of hypoxic brain injury.

Background Abnormalities in left ventricular (LV) structure and function are prevalent in patients on peritoneal dialysis (PD). The risk of cardiovascular mortality is also 10 – 20 times higher in PD patients than in age- and gender-matched healthy control subjects. Methods Echocardiography was performed annually or every 6 months for children on PD; the frequency was increased if the patient had hypertension or any heart problem. We retrospectively reviewed echocardiographic findings of these children (since February 2005) by chart review. The linear regression analysis of log(B-type natriuretic peptide, BNP) was performed to evaluate the association between serum BNP and cardiac parameters on echocardiography. Results We enrolled 52 patients and evaluated 169 echocardiographic findings in association with serum BNP levels. Systolic blood pressure (adjusted R2 = 0.321), diastolic blood pressure (adjusted R2 = 0.292), LV posterior wall thickness at diastole (0.058), LV posterior wall thickness at systole (0.038), LV internal diameter at diastole (0.177), LV internal diameter at systole (0.132), LV mass index (0.122), thickness of interventricular septum at diastole (0.035), the left atrial (LA) diameter (0.201), LA/aorta diameter ratio (0.113), lateral E/E’ (0.229), and lateral A'/E’ (0.149) showed positive correlation with log(BNP); conversely, LV fractional shortening (FS) (0.065), LV ejection fraction (0.082), right ventricular internal diameter at diastole (0.184), right ventricular fractional shortening (0.754), lateral S’ (0.179), and lateral E’ (0.161) showed negative correlation with log(BNP). Conclusions Routine measurement of serum BNP is recommended in children on PD to evaluate the risk of morphological and functional cardiac problems.

BACKGROUND Percutaneous atrial septal defect (ASD) closure is the treatment of choice for patients with a suitable ASD anatomy; however, the procedural characteristics and outcomes in children aged

OBJECTIVES The aims of this study were to evaluate and compare the outcomes after pulmonary valve replacement (PVR) with a mechanical prosthesis (MP) and a bioprosthesis (BP). METHODS From 2004 through 2017, a total of 131 patients, who had already been repaired for tetralogy or Fallot or its variants, underwent their first PVR with an MP or a BP. Outcomes of interests were prosthesis failure (stenosis >3.5 m/s, regurgitation >mild or infective endocarditis) and reintervention. RESULTS The median age at PVR was 19 years. BP and MP were used in 88 (67.2%) and 43 (32.8%) patients, respectively. The median follow-up duration was 7.4 years, and the 10-year survival rate was 96.4%. Risk factors for prosthesis failure were smaller body surface area [hazard ratio (HR) 0.23 per 1 m2, P = 0.047] and smaller prosthesis size (HR 0.73 per 1 mm, P = 0.039). Risk factors for prosthesis reintervention were smaller body surface area (HR 0.11 per 1 m2, P = 0.011) and prosthesis size (HR 0.67 per 1 mm, P = 0.044). Probability of prosthesis failure and reintervention at 10 years were 24.6% (19.5% in BP vs 34.8% in MP, P = 0.34) and 7.8% (5.6% in BP vs 11.9% in MP, P = 0.079), respectively. Anticoagulation-related major thromboembolic events were observed in 4 patients receiving an MP. CONCLUSIONS MP might not be superior to BP in terms of prosthesis failure or reintervention. MP should be carefully considered for highly selected patients in the era of transcatheter PVR.

We investigated the impact of additional antegrade pulmonary blood flow on the long-term outcomes after bidirectional Glenn shunt.From 2001 to 2015, 279 patients underwent bidirectional Glenn shunt as an interim palliation for a functionally single ventricle. After excluding patients with a previous Kawashima or Norwood operation, 202 patients with preexisting antegrade pulmonary blood flow before bidirectional Glenn shunt were included in this study. Antegrade pulmonary blood flow was eliminated in 110 patients (no antegrade pulmonary blood flow group) and maintained in 92 patients (antegrade pulmonary blood flow group). The impact of antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcome was analyzed using inverse probability of treatment weighting.Median age and body weight at bidirectional Glenn shunt were 8 months and 7.8 kg, respectively. Prolonged chest tube drainage or readmission for effusion after bidirectional Glenn shunt was more frequent in the antegrade pulmonary blood flow group (odds ratio, 3.067; 95% confidence interval, 1.036-9.073; P = .043). In the no antegrade pulmonary blood flow group, B-type natriuretic peptide level was decreased further until the Fontan operation (P = .012). In the no antegrade pulmonary blood flow group, oxygen saturation was lower just after bidirectional Glenn shunt, although it was increased further until Fontan operation (P .001), despite still lower oxygen saturation before Fontan operation compared with antegrade pulmonary blood flow group (P .001). The McGoon ratio was decreased in both groups without intergroup difference, although the McGoon ratio before Fontan operation was higher in the antegrade pulmonary blood flow group (2.3 ± 0.4 vs 2.1 ± 0.4, P = .008). Overall transplant-free survival was worse in the antegrade pulmonary blood flow group (hazard ratio, 2.37; confidence interval, 1.089-5.152; P = .030).Maintaining antegrade pulmonary blood flow at bidirectional Glenn shunt was beneficial for higher oxygen saturation and larger pulmonary artery size before Fontan operation. However, it was unfavorable for overall transplant-free survival with a sustained higher risk of death or transplant until the elimination of antegrade pulmonary blood flow.

Biventricular repair and the Fontan operation are irreversible once they are initiated, and they significantly affect the life of the patient. Although 3-dimensional (3D) printing can effectively generate models of complex congenital heart disease (CHD) ([1][1],[2][2]), there is limited evidence

Aim This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis. Methods Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95th percentile value. Differences in numeric data between two datasets were calculated. Results The mean age was 12.9 ± 4.6 years. Predictors of left ventricular hypertrophy and its persistence were systolic blood pressure index (P = 0.019 and P = 0.046) and E' velocity (P = 0.035 and P = 0.031) in univariate analysis. However, differences in these predictors between the datasets were not related to the change in indexed LVMI. Reduction in indexed LVMI was correlated to a reduction of indexed left atrial volume (R = 0.638, P = 0.001), trans-mitral A velocity (R = 0.443, P = 0.011), and serum blood urea nitrogen level (R = 0.372, P = 0.028) and an elevation of haemoglobin level (R = -0.374, P = 0.027). Conclusion The extent of circulating volume expansion is potentially the main predictive factor for change of LVMI, because the volume dependent diastolic functional variables correlate to the change of LVMI. Further study with a large number of ESRD children including a group under fluid volume control is needed to investigate the role of volume expansion on the change of LVMI.

Introduction: Maintaining pulsatility at the time of bidirectional Glenn (BDG) has theoretical advantages and disadvantages. The practice is diverse throughout the centers and individual surgeons without clear rationale behind its application. We sought to investigate the impact of antegrade pulmonary blood flow on pre- and post-Fontan outcomes. Methods: From 2003 through June 2014, among 237 patients who underwent BDG as an interim palliation for functional single ventricle (FSV), patients with preexisting pulmonary blood flow from the ventricle before BDG were included and patients undergoing Kawashima operation or having history of Norwood operation or bilateral pulmonary arterial band were excluded (n=151). Patients were grouped by their presence or absence of antegrade pulmonary blood flow following BDG: group 1 (pulsatile) (n=73) or group 2 (non-pulsatile) (n=78). The pre- and post-Fontan outcomes were compared between two groups. Results: Age and weight at BDG were 7 months and 7.8kg. Oxygen saturation was higher in group 1 (84±4%) compared to group 2 (82±4%) before Fontan (p Conclusions: Maintaining pulsatility at the time of BDG in FSV might be beneficial for pulmonary arterial growth and oxygen saturation without increasing BNP. However, such beneficial effect did not have a positive effect on subsequent Fontan outcome. Attention must be paid to the negative effect of pulsatility on overall post-BDG survival.

Background and Objectives Neo-aortic root dilatation (ARD) and annular dilatation (AAD) tend to develop after arterial switch operation (ASO). However, the trend of neo-aortic growth has not been well established. This paper aims to identify this trend, its associated factors, and predictors of neo-aortic dilatation after ASO. Methods We analyzed the growth trend of the neo-aortic root, annulus, and sinotubular junction (STJ) z-scores using random coefficients model and the risk factors affecting neo-aortic dilatation in 163 patients who underwent ASO from 2006 to 2015. Results Among 163 patients, 41 had a ventricular septal defect, and 11 had Taussig-Bing (TB) anomaly. The median follow-up duration was 6.61 years. The increased in the neo-aortic root z-score was different between the trapdoor and non-trapdoor coronary artery transfer techniques (0.149/year, p

Fetal ventricular tachycardia (VT) is an extremely rare condition. Because sustained VT is highly associated with perinatal morbidity and mortality, prenatal management with antiarrhythmic drugs is crucial. A 33-year-old woman was referred to our hospital for fetal bradycardia at 25.2 weeks of gestation. Initial echocardiography showed bradycardia with irregular atrial beats, which was suspected as blocked atrial bigeminy. Four weeks later, the fetal tachycardia was accompanied by no atrioventricular conduction. Therefore, VT was diagnosed. The fetus developed hydrops. Despite the use of antiarrhythmic drug, the neonate died a few hours after birth because of persistent VT. Only few cases of fetal VT have been reported worldwide, and no case has been reported in Korea. Here, we report a case of fetal VT and discuss the prenatal management using antiarrhythmic drugs, reviewing previous reports.

Transannular patching (TAP) frequently accompanies primary repairs (PRs) in symptomatic neonates with tetralogy of Fallot (TOF). If a systemic-pulmonary shunt (SPS) facilitates the growth of the pulmonary valve annulus (PVA), patients with a marginally small PVA could benefit from a staged repair in terms of lowering the risk of TAP.Among 216 infants with TOF who underwent surgical intervention between January 2004 and December 2013, 29 infants underwent SPS with a subsequent repair (SPS group), whereas 187 infants received a PR (PR group). Median age and the Z-score of the PVA (PVA [Z]) at SPS were 32 days and -3.5, respectively. There was one late death and one follow-up loss after SPS, and preservation of the PVA was achieved on repair in 16 patients (16 of 29; 55%).Multiple regression analysis showed that performance of SPS was the only indicator of the increase in the PVA (Z) in the entire cohort (n = 216). On mixed linear regression, the PVA (Z) increased significantly after the placement of an SPS (-3.6 + 0.2*duration in months, p = 0.001), whereas the prerepair changes in the PVA (Z) were not statistically significant in the PR group (p = 0.7), with a significant intergroup difference (p0.001). Receiver operating characteristic curve analysis showed that placement of TAP is expected when the preshunt PVA (Z) is smaller than -4.2 (area under the curve: 0.82; 95% confidence interval: 0.62 to 1.00; sensitivity, 100%; specificity, 73%).SPS facilitates outgrowth of the PVA over somatic growth in patients with TOF. However, preservation of the PVA may not be achieved on staged repair if the initial PVA is too small.

To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

Purpose: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, labo­ ratory testing was performed more than twice. Results: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequ­ ency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391–2.821; P

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.

Background The aim of study is to identify the dependence of right ventricular (RV) free wall longitudinal deformation on ventricular loading through segmental approach in relatively large number of patients with atrial septal defect (ASD). Methods Patients with ASD (n = 114) and age matched healthy children (n = 60) were echocardiographically examined the day before percutaneous device closure and within 24 hours afterwards. RV free wall deformation parameters, strain (є) and strain rate (SR), were analyzed in the apical (єA, SRA) and basal (єB, SRB) segments. Measured deformation parameters were adjusted for RV size (єAL, SRAL, єBL, SRBL) by multiplying by body surface area indexed RV longitudinal dimension. Regression analyses determined the relationships of these deformation parameters with RV loading parameters that were measured by catheterization. Results єBL and SRBL were not different between pre-closure patients and controls (p = 0.245, p = 0.866), and were decreased post-closure (p = 0.001, p = 0.018). Post-closure єBL was lower than in controls (p = 0.001). Pre-closure єAL and SRAL were higher than in controls (p = 0.001, p < 0.001), but decreased after closure (all p < 0.001). The pulmonary to systemic flow ratio was related to procedural differences of єBL (p = 0.017) and of SRBL (p = 0.019). RV end diastolic pressure was negatively related to post-closure єBL (p = 0.020) and post-closure SRBL (p = 0.012), and the procedural SRBL difference (p = 0.027). Conclusion The longitudinal deformation of the RV basal segment is dependent and its remodeling is also dependent on volume loading in children with ASD.

This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis.Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95The mean age was 12.9 ± 4.6 years. Predictors of left ventricular hypertrophy and its persistence were systolic blood pressure index (P = 0.019 and P = 0.046) and E' velocity (P = 0.035 and P = 0.031) in univariate analysis. However, differences in these predictors between the datasets were not related to the change in indexed LVMI. Reduction in indexed LVMI was correlated to a reduction of indexed left atrial volume (R = 0.638, P = 0.001), trans-mitral A velocity (R = 0.443, P = 0.011), and serum blood urea nitrogen level (R = 0.372, P = 0.028) and an elevation of haemoglobin level (R = -0.374, P = 0.027).The extent of circulating volume expansion is potentially the main predictive factor for change of LVMI, because the volume dependent diastolic functional variables correlate to the change of LVMI. Further study with a large number of ESRD children including a group under fluid volume control is needed to investigate the role of volume expansion on the change of LVMI.

An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2is a quantitative criterion for the diagnosis of Ebstein’s anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein’s anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein’s anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein’s anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2(7.1–119.1 mm/m2) and 7.2 mm/m2(0.0–25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2was applied as a cut-off for the diagnosis of Ebstein’s anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2cannot be used as a cut-off for the diagnosis of complicated Ebstein’s anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

ObjectiveThe objective of this study was to test the hypothesis that limited (

There has been a dramatic increase in the number and type of procedures performed in the field of cardiac intervention in the past decade. Percutaneous intervention is becoming an increasingly recognized modality for the management of prosthetic paravalvular leakages (PVLs) in severely symptomatic non-surgical candidates. Herein, we report our experience of percutaneous closure using the Amplatzer duct occluder for a PVL in a patient who underwent tricuspid valve replacement.

Aortopexy is a surgical procedure in which the aortic arch is fixed at other structures, thus widening the interaortic space. This study aimed to evaluate the outcome of aortopexy by means of chest computed tomography for patients with congenital heart disease. The study retrospectively reviewed the medical records of 16 patients with congenital heart disease who had undergone aortopexy by compressed airway. The severity of compressed bronchus before aortopexy, immediately after aortopexy (≤ 1 month), and after the patient had grown up were compared. To estimate the efficacy of the aortopexy, the interaortic distance index was calculated. Of the 16 patients, aortopexy was performed at the ascending aorta in 7, at the descending aorta in 7, and at the transverse arch in 2. The diameter ratio between the narrowest bronchus and the trachea was improved after aortopexy, as was the diameter ratio before aortopexy versus immediately after aortopexy (n = 9; p = 0.018) and the diameter ratio before aortopexy versus after the patient had grown up (n = 11; p = 0.006). Also, the interaortic distance index was increased after aortopexy, as was the diameter before aortopexy versus immediately after aortopexy (n = 9; p = 0.039) and the diameter before aortopexy versus after the patient had grown up (n = 11; p = 0.014). The study had one case of mortality due to sepsis. As shown by the results, aortopexy in patients with a compressed airway between arches is a useful surgical option. Such a compressed airway between arches should be considered for patients with an unusual clinical course before and after open heart surgery.

Transcatheter treatment of aortic coarctation, with balloon angioplasty or stent implantation, is now an acceptable alternative to surgical repair. However these procedures may result in complications, such as vascular wall injury and re-stenosis of the lesion. A nitinol self-expandable stent, when deployed at the coarctation site, produces low constant radial force, which may result in a gradual widening of the stenotic lesion leaving less tissue injury ('stretching rather than tearing'). For an adolescent with a native aortic coarctation, a self-expandable stent of 20 mm diameter was inserted at the discrete stenotic lesion of 5 mm diameter without previous balloon dilatation procedure. No further balloon dilatation was done immediately after the stent insertion. With the self-expandable stent only, the stenosis of the lesion was partially relieved immediately after the stent deployment. Over several months after the stent insertion, gradual further widening of the stent waist to an acceptable dimension was observed.

Although several previous studies have reported that a ventricular septal defect (VSD) can be closed safely through the detached tricuspid valve (TV) without impairing TV function, this has not been demonstrated for infants younger than 3 months.We enrolled 296 infants younger than 3 months who underwent VSD closure through the right atrium between January 2004 and December 2013. In the study group of 49 patients (16.6%), the TV was detached for better exposure of the defect.The median age and weight were 63.5 days (14-90 days) and 4.4 kg (2.1-6.7 kg), without intergroup differences. Abnormal chordal attachment of the TV in preoperative echocardiography was associated with detachment of the TV during surgery (P = .001). Cardiopulmonary bypass (P = .001) and aortic crossclamp (P .001) times were significantly longer in the study group. A permanent pacemaker was required for 2 patients in the control group. Follow-up echocardiography was available for 282 patients at a median of 21 months (1-108 months) after the operation. On final echocardiography, tricuspid regurgitation greater than grade 2 was observed in 1 patient in the study group and residual VSD existed in 4 patients (1 in the study group), without statistical significance.Detachment of the TV can be used safely for better exposure of the VSD without increased risk of tricuspid regurgitation in infants younger than 3 months. Preoperative echocardiography might be useful for predicting the possibility of detachment of the TV for enhancing exposure of the VSD.

Objectives The objectives of this study were to evaluate outcomes of pulmonary valve replacement (PVR) in patients with chronic pulmonary regurgitation (PR) and to better define the optimal timing of PVR. Background Although PVR is effective in reducing right ventricular (RV) volume overload in patients with chronic PR, the optimal timing of PVR is not well defined. Methods A total of 170 patients who underwent PVR between January 1998 and March 2011 for chronic PR were retrospectively analyzed. To define the optimal timing of PVR, pre-operative and post-operative cardiac magnetic resonance imaging (MRI) data (n = 67) were analyzed. Results The median age at the time of PVR was 16.7 years. Follow-up completeness was 95%, and the median follow-up duration was 5.9 years. Overall and event-free survival at 10 years was 98% and 70%, respectively. Post-operative MRI showed significant reduction in RV volumes and significant improvement in biventricular function. Receiver-operating characteristic curve analysis revealed a cutoff value of 168 ml/m 2 for non-normalization of RV end-diastolic volume index (EDVI) and 80 ml/m 2 for RV end-systolic volume index (ESVI). Cutoff values for optimal outcome (normalized RV volumes and function) were 163 ml/m 2 for RV EDVI and 80 ml/m 2 for RV ESVI. Higher pre-operative RV ESVI was identified as a sole independent risk factor for suboptimal outcome. Conclusions Midterm outcomes of PVR in patients with chronic PR were acceptable. PVR should be considered before RV EDVI exceeds 163 ml/m 2 or RV ESVI exceeds 80 ml/m 2 , with more attention to RV ESVI.

Total correction of tetralogy of Fallot (TOF) is typically performed within the first year of life. However, subsequently, right-ventricular dysfunction often progresses by pulmonary stenosis or regurgitation. Although most patients with corrected TOF require additional surgery for right-ventricular outflow tract, intervention could be an alternative to palliative surgery and associated morbidities, especially in children. We describe a palliative application of the self-expandable stent in a patient with pulmonary stenosis after total correction of TOF who lacked a conduit between the right ventricle and pulmonary artery.