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Purpose: Atrial flutter is an uncommon arrhythmia in the neonatal period. This study aimed to describe the cause and clinical course of atrial flutter in neonates.Methods: The medical records of 14 patients diagnosed with atrial flutter at Ulsan University Hospital Neonatal Intensive Care Unit (NICU) between March 2008 and August 2020 were reviewed retrospectively.Results: All 14 cases occurred on the first day of birth. Of these, two were term infants, and 12 were preterm infants. Causes of atrial flutter included three cases of the umbilical venous catheter misplacement, one with a diabetic mother, and one ivolving atrial flutter after an intravenous aminophylline injection. Thirteen patients had structurally normal hearts with no congenital heart diseases. The patient, born to a diabetic mother, had an atrial septal defect and ventricular hypertrophy. Adenosine was administered first to differentiate it from paroxysmal supraventricular tachycardia. Synchronized cardioversion was attempted in 11 patients, while one received it after an esmolol injection that failed to convert to sinus rhythm. One patient had a recurrence after the intrusion of a peripherally inserted central catheter; however, atrial flutter disappeared after repositioning it. No patient had a recurrence after discharge.Conclusion: Neonatal atrial flutter is a rare tachyarrhythmia with the risk factors often unknown; however, it could occur in structural heart disease, mispositioning of the umbilical venous catheter, and if the mother has diabetes. During umbilical venous catheterization, clinicians should be cautious and ensure appropriate monitoring of infants in the NICU as it may cause complications.

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.

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.

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

Graft-versus-host disease (GVHD) is related to considerable morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT). Cardiac complications associated with GVHD are uncommon, and coronary artery involvement is even more unusual. We report on a male pediatric patient with chronic GVHD who developed a fatal ventricular arrhythmia caused by coronary artery obstruction after HSCT. At 30 months after HSCT, he suddenly collapsed with ventricular fibrillation. After resuscitation, electrocardiography showed abnormal q-wave and ST changes in the inferior leads, suggesting a coronary event. Coronary angiography revealed complete obstruction of the proximal left anterior descending artery, subtotal obstruction of the mid left circumflex artery, and mild narrowing at the right coronary artery. This boy had none of the risk factors for coronary artery disease, and the only possible explanation for the cardiac event is GVHD. Coronary artery disease only rarely occurs as a cardiac event in children. However, coronary artery involvement should be recognized as one of the important manifestations of chronic GVHD in children.

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.

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

Background and Objectives Transcatheter device closure becomes the first option for treating secundum atrial septal defect (ASD), but the conventional method is sometimes unsuccessful even when the defect size indicates the closure to be feasible. To increase the success rate, modified methods have been introduced and used. This study aimed to find predictors for using the modified methods in the device closure of secundum ASDs. Subjects and Methods Between October 2010 and December 2012, 92 patients with ASDs underwent the transcatheter device closure. We analyzed the sizes of the defect, the surrounding rims, and the ratios of the left atrium (LA) dimensions to the device size in the patients who underwent the procedure either using the conventional or modified methods. Results Among the 88 successful cases (95.7%), 22 patients (25%) required modified methods (12 using pulmonary vein and 10 using balloon). The modified method group had the larger size of ASDs and smaller posterosuperior rim. The mean ratios of the LA anteroposterior diameter, width, and length to the device size were all significantly smaller in the modified methods group than in the conventional group (1.20 vs. 1.56, 1.32 vs. 1.71, and 1.61 vs. 2.07, respectively). We found that the risk factors for the modified methods were smaller retroaortic rim, larger ASD, and smaller LA dimension/device size. Conclusion In addition to larger defects and smaller retroaortic rim, the smaller ratios of the LA dimensions to the device size influenced the need for the application of modified methods in the transcatheter device closure of ASDs.

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.

OBJECTIVES: Systemic hypertension is the main late complication after arch reconstruction in patients with arch obstruction. Gothic arch geometry is suspected to be one of its possible causes. Accordingly, we evaluated here if a modified arch repair technique using an autologous pulmonary patch is effective in preventing gothic arch development. METHODS: Fifty infants who underwent arch repair with either a modified (n= 17) or conventional (n= 33) technique between January 2006 and August 2012 by a single surgeon were retrospectively reviewed. Arch geometry was compared using three categories (gothic, crenel or roman), classified by the height/width (H/W) ratio and the arch angle measured in computed tomography. RESULTS: No gothic arch geometry was observed in the modified group, whereas it was observed in 9 cases in the conventional group (P= 0.005). Moreover, reintervention for arch restenosis was performed only in the conventional group (n =4 ;P= 0.29). No associated complications were observed, although the selective cerebral perfusion time was longer in the modified group than in the conventional group (28.5 ± 6.2 vs 17.1 ± 9.9 min; P< 0.001). Otherwise, there were no significant differences in clinical variables between the groups. The mean follow-up duration was 55.3 ± 26.7 months. Significant systemic hypertension was not observed in our study cohort. CONCLUSIONS: Our modified technique was proven to be not only highly effective in preventing gothic arch geometry, but also as equally safe in terms of early clinical outcomes as conventional arch reconstruction techniques.

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.

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.

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.

Left ventricular (LV) dysfunction and dilated cardiomyopathy (DCM) are rarely attributable to sustained or incessant tachyarrhythmias in infants and children with Wolff-Parkinson-White (WPW) syndrome. However, several recent reports suggested that significant LV dysfunction may develop in WPW syndrome in the absence of tachyarrhythmias. It is assumed that an asynchronous ventricular activation over the accessory pathway, especially right-sided, induces septal wall motion abnormalities, ventricular remodeling and ventricular dysfunction. The prognosis of DCM associated with asymptomatic WPW is excellent. Loss of ventricular pre-excitation results in mechanical resynchronization and reverse remodeling where LV function recovers completely. The reversible nature of LV dysfunction after loss of ventricular pre-excitation supports the causal relationship between LV dysfunction and ventricular pre-excitation. This review summarizes recent clinical and electrophysiological evidence for development of LV dysfunction or DCM in asymptomatic WPW syndrome, and discusses the underlying pathophysiological mechanism.

OBJECTIVE: Regarding neo-aortic valve regurgitation (neo-AR) after the arterial switch operation (ASO), the ‘trap-door’ technique was supposed to be a risk factor due to a distortion of the sinotubular junction (STJ) geometry. Here we report our results of the ‘trap-door’ technique with a special emphasis on root geometry including the ratio of STJ to annulus. METHODS: From August 1991 to March 2010, 240 patients with transposition of the great arteries underwent the ASO and who had at least 1 year of follow-up were included in this study. The medical records were retrospectively reviewed. RESULTS: The median age and body weight at the time of operation were 11 (0–1213) days and 3.4 (1.30–18.75) kg, respectively. The median follow-up duration was 79 months (range 12 months—19.5 years). At the latest echocardiographic follow-up, only six patients had neo-AR greater than Grade II (6 of 240, 2.5%). We found no relationship between neo-AR greater than Grade II and perioperative factors. The actual sizes of the neo-aortic annulus, mid-sinus and STJ were observed as having increased over time. However, most zscores of STJ at the latest echocardiography varied between �2 and 2 and, more importantly, the ratio of STJ to neo-aortic annulus was 0.93 ± 0.20, which was near normal at the latest echocardiographic follow-up. CONCLUSIONS: Our results showed a very low incidence of significant neo-AR, which was relatively attributable to the preserved z-score of STJ and the normal range of STJ/annulus ratio. Therefore, we propose that it is important to maintain these factors adequately during the reconstruction of the neo-aortic root in the ASO.

Background and Objectives: Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. Subjects and Methods: A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. Results: Percent stenosis of stenotic lesions were decreased from 54.1±10.7% to 22.8±12.5% (p

PURPOSE Brain natriuretic peptide (BNP) has been considered a biochemical marker for myocarditis in Kawasaki disease. We performed this study to determine its quantitative significance. METHODS We attempted to correlate log-transformed BNP concentrations (log-BNP) and clinical, laboratory, and echocardiographic variables in 81 children with Kawasaki disease. Stepwise multiple linear regression analysis was used to determine the variables independently associated with log-BNP concentration. RESULTS Serum C-reactive protein level (P

One and a half ventricle repair (1½ repair) strategy has been used for patients with a hypoplastic or dysfunctional right ventricle (RV), or both. We sought to assess the postoperative hemodynamics of 1½ repair using phase-contrast magnetic resonance imaging (PC-MRI).Ten adults, 9 with Ebstein's anomaly and 1 with tricuspid stenosis, underwent 1½ repair (median age at operation, 42.4 years). The azygos vein was left open in all patients on 1½ repair to prevent severe postoperative central venous hypertension. Postoperative PC-MRI studies were performed to measure blood flow to the ascending aorta (QAsc-Ao), the main pulmonary artery (QMPA), the superior vena cava (SVC) (QSVC), and the branch pulmonary arteries and veins. From these values, blood flow to the upper compartment of the body (QUC), right ventricular volume unloading effect (QMPA/QAsc-Ao), proportion of blood flow to the upper compartment of the body (QUC/QAsc-Ao), and venous return to arterial forward flow ratio of the upper compartment of the body (QSVC/QUC) were calculated. Two patients also underwent preoperative PC-MRI.On PC-MRI, QMPA/QAsc-Ao, QUC/QAsc-Ao, and QSVC/QUC were 0.58 to 0.84 (median, 0.67), 0.19 to 0.36 (median, 0.27), and 0.47 to 1.57 (median, 0.93, lower than 1.0 in 7 patients), respectively. In 2 patients who had preoperative and postoperative PC-MRI, QUC/QAsc-Ao decreased from 0.26, 0.32 to 0.21, 0.28, respectively.After 1½ repair, right ventricular volume unloading was effective in all patients, but intercaval collateral veins (ie. QSVC/QUC1) appeared to develop in most of the patients. Furthermore blood flow to the upper compartment of the body appeared to diminish, presumably due to postoperative elevation of central venous pressure.

Objective Tetralogy of Fallot (ToF) with infundibular septal deficiency (ISD) is commonly associated with postoperative right ventricular outflow tract obstruction (RVOTO), presumably caused by subpulmonary extension of ventricular septal defect (VSD) and restricted growth of the pulmonary valve annulus (PVA). We sought to determine the postoperative growth of the PVA after annulus preservation according to the presence or absence of the infundibular septum. Methods A retrospective review of 90 patients who underwent surgical repair of ToF between June 1997 and August 2008 was performed. Median age at operation was 9.5 months. Infundibular septum was absent in 15 (15/90, 17%). PVA was preserved in 71 patients (71/90, 79%), including 13 patients with ISD (13/15, 87%). Results Median follow-up duration was 39 months (2 months to 13 years). There was no early mortality and one late noncardiac death. Among the patients with PVA preservation, reoperation for RVOTO was performed in 4 patients (4/71, 6%), including 3 patients without the infundibular septum. ISD was identified as the only risk factor in reoperation for RVOTO after PVA preservation (RR: 21.85, P = .007). Among the patients who underwent repair with PVA preservation during infancy (n = 43), PVA (Z-score) increased postoperatively in patients with the infundibular septum (+ 0.021/month, P = .009), whereas the changes in PVA (Z-score) were nonsignificant in patients with ISD (-0.021/month, P = .306), with a marginal intergroup difference ( P = .056). Conclusions PVA preservation in ToF with ISD may be associated with a higher risk for postoperative RVOTO, which can be attributed to the restricted growth of the PVA.

Although the outcome of neonatal cardiac surgery has dramatically improved, low body weight (LBW) is still considered an important risk for open heart surgery. The factors contributing to poor outcomes in LBW infants, however, are still unclear. We investigated risk factors for poor outcomes in infants weighing

Background Preoperative risk analysis for Fontan candidates is still less than optimal in that patients with apparently low risks may have a poor outcome, such as prolonged pleural drainage, protein-losing enteropathy, pulmonary thromboembolism and death. We hypothesized that low pulmonary vascular compliance (PVC) is a risk factor for persistent pleural effusion after the Fontan operation. Methods A retrospective review of 85 patients who underwent the extracardiac Fontan procedures (median age: 3.87 years) was performed. Fontan risk score (FRS) was calculated from 12 categorized preoperative anatomical and physiological variables. PVC (mm 2 /m 2 ·mmHg) was defined as pulmonary artery index (mm 2 /m 2 ) divided by total pulmonary resistance (Wood Unit·m 2 ) and pulmonary blood flow (L/min/m 2 ), based on the electrical circuit analogy of the pulmonary circulation. Chest tube indwelling time was log-transformed (log indwelling time, LIT) to fit normal distribution, and the relationship between perioperative predictors and LIT was analyzed by multiple linear regression. Results Preoperative PVC, chest tube indwelling time and LIT ranged from 6 to 94.8 mm 2 /mmHg/m 2 (median: 24.8), 3 to 268 days (median: 20 days), and 1.1 to 5.6 (mean: 2.9, standard deviation: 0.8), respectively. FRS, PVC, cardiopulmonary bypass time (CPB) and central venous pressure at postoperative 12 h were correlated with LIT by univariable analyses. By multiple linear regression, PVC ( p =0.002) and CPB ( p =0.003) independently predicted LIT, explaining 22% of the variation. The regression equation was LIT=2.744−0.016 PVC+0.007 CPB. Conclusion: Low pulmonary vascular compliance is an important risk factor for prolonged pleural effusion drainage after the extracardiac Fontan procedure.

In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease.

Plastic bronchitis is a rare cause of acute obstructive respiratory failure in children. Life-threatening events are much more frequent in patients with repaired cyanotic congenital heart disease, and most frequent following a Fontan operation. Commonly, the diagnosis is not made until bronchial casts are expectorated. Detailed CT findings in plastic bronchitis have not been described.To describe the CT findings in plastic bronchitis in children after a Fontan operation.Three children with plastic bronchitis after a Fontan operation were evaluated by chest CT. Bronchial casts were spontaneously expectorated and/or extracted by bronchoscopy. Airway and lung abnormalities seen on CT were analyzed in the three children.CT demonstrated bronchial casts in the central airways with associated atelectasis and consolidation in all children. The affected airways were completely or partially obstructed by the bronchial casts without associated bronchiectasis. The airway and lung abnormalities rapidly improved after removal of the bronchial casts.CT can identify airway and lung abnormalities in children with plastic bronchitis after a Fontan operation. In addition, CT can be used to guide bronchoscopy and to monitor treatment responses, and thereby may improve clinical outcomes.

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.

Background: Recently, several studies to predict the unresponsiveness to initial intravenous immunoglobulin (IVIG) treatment during the acute phase of Kawasaki disease have been conducted. However, reported predictors are varied in those studies. The racial difference was assumed as the cause of such diversity. The aim of this study is to find predictors of unresponsiveness to initial IVIG in Korean children with Kawasaki disease. Subjects and methods: Subjects were 424 patients with Kawasaki disease who had been admitted to Asan Medical Center from January 2006 to December 2012. Demographic, clinical, and laboratory data were investigated through a review of medical records. Laboratory data before initial IVIG treatment and within 10 days after fever onset were selected. Forty one patients (9.7%) were non responders to initial IVIG treatment. Univariate and multivariate logistic regression analyses were performed to find predictors of unresponsiveness to initial IVIG treatment. Results: Median age was 30.0 months (range 1.6-186.0 months) in IVIG-responders, and 30.9 months (range 2.9-107.8 months) in IVIG-nonresponders (P=0.819). Number of females was 156 (40.7%) and 15 (36.6%) respectively (P=0.607). No other demographic and clinical variables were different between two groups. Univariate analyses showed that neutrophil percentage (P Conclusion: The results of this study were inconsistent with the results of previously conducted studies with the same purpose in other countries. Therefore future study with more number of subjects is needed for confirmative identification of predictors of unresponsiveness to initial IVIG treatment in patients with Kawasaki disease.

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.

Although the incidence of coronary artery aneurysms has diminished in patients with Kawasaki disease, coronary artery involvement is still regarded as a major complication of the disease, significantly affecting morbidity and mortality. Recent technical advances in coronary CT angiography (CTA) and MR angiography (MRA) have led to the possibility of using these two imaging methods as minimally invasive alternatives to the more invasive diagnostic catheter angiography in evaluating coronary artery abnormalities, such as aneurysm, stenosis, and occlusion. In this article, we describe imaging techniques and findings of coronary CTA and MRA in Kawasaki disease.

Atrioventricular reciprocating tachycardia (AVRT) using an accessory pathway is the most common supraventricular tachycardia observed in infancy and childhood. There is a general agreement to treat children older than 5 years who are on a potentially long-term antiarrhythmic agent with radiofrequency catheter ablation. Atenolol, a relatively long-acting and cardioselective beta-adrenoceptor blocker, has been used to control the various types of supraventricular tachycardia in children and adults. There are few reports on the use of atenolol in children

Transesophageal atrial pacing study was used to document arrhythmias in 67 infants and children age 2 months to 16 years (mean, 8.3 years), who had palpitations or symptoms suggesting tachyarrhythmias but had no electrocardiographic documentation of cardiac dysrhythmias. The transesophageal pacing and medical records were reviewed retrospectively. In 47 of 67 (70%) of the infants and children with suspected tachyarrhythmias, transesophageal atrial pacing induced various tachycardias, which may be the cause of symptoms. In 10 of 67 patients, tachycardia was induced during infusion of isoproterenol. During the study, tachycardia was initiated in 14 of 15 patients less than6 years-old and in 33 of 52 patientsor = 6 years-old (P0.05). Of these induced tachycardias, 25 of 47 were atrioventricular reciprocating tachycardia, 16 atrioventricular nodal reentrant tachycardia, and 6 idiopathic left ventricular tachycardia. Both transesophageal study and invasive electrophysiologic study were performed in 10 patients. The mechanisms of tachycardia in the invasive study and transesophageal study were identical except for one patient. In conclusion, transesophageal atrial pacing and recording was less invasive, safe and useful for documenting arrhythmias in infants and children who had symptoms suggesting tachyarrhythmias, especially in patients6 years of age. Evaluation of the mechanism of induced tachycardia provided useful information regarding the prognosis and therapeutic options in infants and children.

Computed tomography (CT) plays an important supplementary role in the evaluation of patients with congenital heart disease (CHD). Fast multisection spiral CT can be used to obtain isotropic volume data, and high-quality two- and three-dimensional multiplanar reformatted images can be created to accurately and systematically delineate the normal and pathologic morphologic features of the cardiovascular system. CT may be technically challenging and demanding in uncooperative young children. However, it can be used to systematically evaluate the aorta, pulmonary artery, pulmonary vein, cardiac chambers and ventriculoarterial connection, relationship between the upper lobe bronchi and pulmonary arteries, coronary artery, valves, systemic veins (superior vena cava, inferior vena cava, hepatic veins), and visceral situs with a step-by-step approach. This approach may be helpful in understanding the anatomy of the cardiovascular system in CHD patients. CT has both advantages and disadvantages in evaluating patients with CHD. Nevertheless, it is useful in this setting, and radiologists who perform CT in young children with CHD should be familiar with the advantages and disadvantages of CT and with the normal anatomy and typical pathologic conditions in affected patients.

목적: 가와사키병이 있는 소아에서 진단 및 결과 예측에 있어서 Bacille Calmette-Guerin (BCG) 접종 부위 반응의 임상적 중요성을 알아보고자 하였다. 방법: 2006년 1월부터 2017년 2월까지 가와사키병으로 서울아산병원에 입원한 1,058명의 환자를 대상으로 각 연령대에서 BCG 부위 반응의 발생 빈도를 조사하였다. 이 중 생후 18개월 미만인 416명의 환자를 BCG 부위 반응과 실험실 및 임상 소견들과의 연관성을 분석하기 위한 대상자들로 선정하였다. 분석은 완전형과 불완전형 발현 두 그룹으로 구분하여 수행되었다. 결과: 1,058명의 환자들을 관찰한 결과, BCG 부위 반응의 발생률은 생후 6-12개월(83%) 연령의 환자에게 가장 많았고, 12개월 후 연령이 증가함에 따라 감소했다(P

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.

Horseshoe lung is a rare congenital pulmonary anomaly of childhood that can be accompanied with other anomalies. The diagnosis has historically been accomplished with invasive catheter angiography and bronchography rather than CT. Two infants with horseshoe lung were recently diagnosed with CT. We report the imaging findings in these two patients with emphasis on angiographic and bronchographic demonstration of key abnormalities of horseshoe lung using multidetector-row spiral CT.

Purpose The aim of this study was to investigate the statistical properties of four previously developed pediatric coronary artery z score models in healthy Korean children. Methods The study subjects were 181 healthy Korean children, whose age ranged from 1 month to 15 years. The diameter of each coronary artery was measured using 2-dimensional echocardiography and converted to the z score in the four models (McCrindle, Olivieri, Dallaire, and Japanese model). Descriptive statistical analyses and 1-sample t tests were performed. Results All calculated z scores had P values of ≥0.050 using the Kolmogorov-Smirnov test. The one sample t test showed that the mean z scores did not converge to zero except in 1 model, and the mean right coronary artery (RCA) z score was less than zero in all 4 models. The smaller RCA diameter in this study could be associated with the more distal measuring point used to avoid the conal branch. The percentage of subjects with extreme z score values (≥2.0 and ≥2.5) for the left main coronary artery (LMCA) seems to be higher in the Dallaire (4.9% and 3.3%) and Japanese models (7.1% and 3.8%). Conclusion All 4 models showed statistical feasibility of normal distribution. More precise instructions would be needed for the measurement of the RCA. The higher percentage of extreme z scores for the LMCA is compatible with the basic understanding of anatomic variation in the LMCA.

Ebstein’s anomaly is frequently detected before birth, with prenatal detection accounting for the majority of cases in the current population. This study aimed to identify the outcome variables among these infants. The medical records of 59 patients with neonatal Ebstein’s anomaly managed at the Asan Medical Center between January, 2001 and June, 2012 were investigated retrospectively. In 46 cases, the diagnosis was made prenatally. Surgical/interventional procedures were performed for 27 of the analyzed patients. Biventricular repair was successful for 12 patients but not for 9 patients with pulmonary atresia. The median follow-up period was 1.96 years (range 0.0–10.4 years). The overall mortality rate was 23.7 % (14/59). Of the 14 deaths, 5 occurred within several hours after birth. The 1- and 5-year survival rates were 78.6 and 76.3 %, respectively. Univariate analysis identified several variables related to the time to death: fetal distress (p = 0.002), prematurity (p = 0.036), low birth weight (p = 0.003), diameter of the atrial septal defect (p = 0.022), and pulmonary stenosis/atresia (p = 0.001). Neither the Carpentier classification (p = 0.175) nor the Celermajer index (p = 0.958) was a significant variable. According to the multivariate analysis, fetal distress (p = 0.004) and pulmonary atresia/stenosis (p < 0.001) were significant determinants of outcome. In conclusion, fetal distress and pulmonary atresia/stenosis are significant predictors of mortality in the current population of patients with neonatal Ebstein’s anomaly. A close cooperation of associated clinicians is required for an improvement in outcome. To establish a better surgical strategy for patients with Ebstein’s anomaly and pulmonary atresia, studies of larger populations are required.

Background Homografts or bioprosthetic valves have been preferred in the pulmonic position in patients with congenital heart disease. However, unsatisfactory long-term results have aroused interest in the use of mechanical valves. In this study, we investigated the long-term outcomes of mechanical valves implanted in the pulmonic position. Methods The medical records of 37 patients (27 male, 73%) who underwent 38 mechanical pulmonary valve replacements between October 1988 and February 2011 were reviewed, retrospectively. The median age of patients was 13.5 years (range, 7 months to 23 years), and the median number of prior operations per patient was 2 (range, 0 to 5). Tetralogy of Fallot was the most common diagnosis (n = 23). The median valve size was 23 mm (range, 17 to 27 mm), and the median follow-up duration after pulmonary valve replacement was 24.6 months (range, 1.3 months to 22.5 years). Events were defined as the following: valve failure, thrombosis, embolism, bleeding, reoperation, and death. Results There was no in-hospital mortality, but there were 2 late deaths (1 heart failure and 1 traffic accident at 10.8 months and 8.7 years postoperatively, respectively). Excluding the traffic accident death, survival rates were 97%, 97%, and 97%, at 1, 5, and 10 years, respectively. Freedom from thromboembolism or bleeding events was 92%, 92%, and 78.8%, at 1, 5 and 10 years, respectively. Two reoperations were performed at 6.8 and 10.2 years postoperatively. Freedom from reoperation was 100%, 100%, and 85.7%, at 1, 5, and 10 years, respectively. Conclusions Durability of mechanical valve in pulmonic position was excellent. Thromboembolism or bleeding events due to anticoagulation therapy were rare. In growing patients who have undergone prior sternotomies requiring a pulmonary valve replacement, a mechanical valve could be an attractive option.