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To treat congenital heart disease, it is important to understand the anatomical structure correctly. Three-dimensional (3D) printed models of the heart effectively demonstrate the structural features of congenital heart disease. Occasionally, the exact characteristics of complex cardiac malformations are difficult to identify on conventional computed tomography, magnetic resonance imaging, and echocardiography, and the use of 3D printed models can help overcome their limitations. Recently, 3D printed models have been used for congenital heart disease education, preoperative simulation, and decision-making processes. In addition, we will pave the way for the development of this technology in the future and discuss various aspects of its use, such as the development of surgical techniques and training of cardiac surgeons.

Kawasaki disease (KD) is an acute, self-limited vasculitis, mainly affecting children younger than 5 years old, with accompanying fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) is the standard treatment for KD; however, ~15% of patients are resistant to IVIG treatment. To identify protein coding genetic variants influencing IVIG resistance, we re-analyzed our previous genome-wide association study (GWAS) data from 296 patients with KD, including 101 IVIG non-responders and 195 IVIG responders. Five nonsynonymous SNPs (nsSNPs) in five immune-related genes, including a previously reported SAMD9L nsSNP (rs10488532; p.Val266Ile), were associated with IVIG non-response (odds ratio [OR] = 1.89–3.46, P = 0.0109–0.0035). In a replication study of the four newly-identified nsSNPs, only one in the interleukin 16 (IL16) gene (rs11556218, p.Asn1147Lys) showed a trend of association with IVIG non-response (OR = 1.54, P = 0.0078). The same IL16 nsSNP was more significantly associated with IVIG non-response in combined analysis of all data (OR = 1.64, P = 1.25 × 10−4). Furthermore, risk allele combination of the IL16 CT and SAMD9L TT nsSNP genotypes exhibited a very strong effect size (OR = 9.19, P = 3.63 × 10−4). These results implicate IL16 as involved in the mechanism of IVIG resistance in KD.

Purpose Recognition of cardiogenic syncope caused by acute myocarditis masquerading as febrile seizures (FS) in children can be difficult in the emergency department (ED) before a cardiac work-up. We aimed to identify clinical and laboratory characteristics of children with seizure-like activity and fever caused by myocarditis that would enable their condition to be distinguished from benign FS. Methods We identified seven children who visited the ED for paroxysmal seizure-like activity with fever and were diagnosed with acute myocarditis between 2012 and 2015, as well as 204 children who were diagnosed with benign FS during the same period. A detailed retrospective review of the medical charts of both groups was conducted. Results Age at onset of seizure-like activity was much higher in the myocarditis group than in the FS group (4.4±1.9 years vs. 2.4±1.1 years, P=0.033). Body temperature at seizure-like activity onset was significantly lower in the myocarditis group than in the FS group (37.9°C±0.2°C vs. 38.7°C±0.6°C, P

Background The aim of this study was to determine if there was a difference between coronary reimplantation after neoaortic reconstruction and open coronary reimplantation technique in arterial switch operation (ASO). Methods A total of 236 patients who underwent ASO from March 1994 to August 2018 were enrolled in this study. Multivariate analysis was performed for postoperative early mortality. Patients were divided into the open coronary reimplantation and coronary reimplantation after neoaortic reconstruction groups. The 30-day mortality, intraoperative and postoperative coronary artery (CA) revisions, CA–related late morbidity and mortality, and early and late neoaortic valve regurgitations after ASO were compared between the two groups. Results Overall postoperative early mortality was 7.2% (17/236). Patients who underwent open coronary reimplantation had higher early mortality as compared with those who underwent coronary reimplantation after neoaortic reconstruction. Risk factors for postoperative early mortality from multivariate analysis were cardiopulmonary bypass time and open coronary reimplantation. There was a higher incidence of CA–related late mortality or morbidity in the open coronary reimplantation group. The open coronary reimplantation group had a higher incidence of intraoperative or postoperative CA revision. There were no differences in the incidence of mild or more neoaortic valve regurgitation at discharge or in the 5-year freedom from mild or more neoaortic valve regurgitation. Conclusions CA reimplantation after neoaortic reconstruction yields better results in mortality and intraoperative or postoperative CA–related problems in ASO without increasing postoperative neoaortic valve regurgitation.

Purpose Prognostic factors of coronary aneurysms in Kawasaki disease have been investigated in many studies. The aim of this study was to identify risk factors associated with early and late coronary artery outcomes in treated patients with Kawasaki disease. Methods A total of 392 patients diagnosed with Kawasaki disease from January 2012 to December 2015 in Pusan National University Children's Hospital were retrospectively selected as subjects of the present study to determine risk factors for coronary aneurysms and persistence of coronary aneurysms after a 1-year follow-up. Results Coronary aneurysms were detected in 30 of 392 patients within 1 month after the occurrence of Kawasaki disease. Coronary aneurysms persisted in 5 of 30 patients after a 1-year follow-up. A long duration of fever (adjusted odds ratio [OR], 1.47; 95% confidence interval [CI], 1.06-2.02; P=0.018) and high platelet count (adjusted OR, 1.00; 95% CI, 1.00-1.01; P=0.009) were found to be independent factors to predict the development of coronary aneurysms in the early phase. Initial coronary severity (adjusted OR, 46.0; 95% CI, 2.01-1047.80; P=0.016) and a high white blood cell count (adjusted OR, 1.17; 95% CI, 1.01-1.36; P=0.028) were found to be significant factors for the persistence of late coronary aneurysms in univariate analysis. However, no significant factors were found in multivariate analysis. Conclusion These data showed early and late follow-up of coronary aneurysms in our unit. Further studies are needed to determine the mechanisms involved in the disappearance of coronary aneurysms and related factors.

Kawasaki disease (KD) is a systemic vasculitis affecting infants and children; it manifests as fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) treatment effectively attenuates the fever and systemic inflammation. However, 10–20% patients are unresponsive to IVIG. To identify genetic variants influencing IVIG non-response in KD, a genome-wide association study (GWAS) and a replication study were performed using a total of 148 IVIG non-responders and 845 IVIG-responders in a Korean population. rs28662 in the sterile alpha motif domain-containing protein 9-like (SAMD9L) locus showed the most significant result in the joint analysis of GWAS and replication samples (odds ratio (OR) = 3.47, P = 1.39 × 10−5). The same SNP in the SAMD9L locus was tested in the Japanese population, and it revealed a more significant association in a meta-analysis with Japanese data (OR = 4.30, P = 5.30 × 10−6). These results provide new insights into the mechanism of IVIG response in KD.

Coronary reimplantation after neoaortic reconstruction (CRANR) in the arterial switch operation (ASO) allows easy selection of accurate coronary transfer sites in the distended neoaorta. However, neoaortic valve injury may occur during coronary reimplantation. We determined whether the CRANR procedure increased the incidence of aortic valve regurgitation (AR) after ASO. Between March 1994 and August 2017, 227 patients underwent ASO. Since September 2000 CRANR has been performed on 155 patients and open coronary reimplantation (OCR) on 72. Patients who had undergone aortocoronary flaps procedures (n = 13), had early or late mortality (n = 27), or lacked data (n = 11) were excluded. We enrolled and retrospectively reviewed the medical records of 176 patients who were followed up for postoperative AR: 38 underwent OCR and 138 underwent CRANR. We compared the incidences of early and late postoperative AR in both groups. We defined mild or greater AR as "significant AR." The groups did not differ in body weight at operation, great artery relationship, and coronary artery anatomy. The incidences of significant AR at discharge were 21.1% (8/38) in the OCR group and 16.6% (23/138) in the CRANR group (p = 0.53). The freedom from significant AR at 5 years was 59.9% in the OCR group and 62.4% in the CRANR group with no difference between the two groups (p = 0.73). Moderate AR occurred in one patient in the CRANR group. No surgical intervention was required for the aortic valve in either group. ASO using the CRANR technique did not increase the incidence of postoperative early and late AR.

Background: Left pulmonary vein (PV) obstruction can occur due to compression between the left atrium (LA) and the descending aorta (DA). One of the effective solutions for this problem is posterior aortopexy. In this study, we have reported five cases of posterior aortopexy to relieve left PV obstruction between the LA and the DA. Methods: Since August 2012, five patients have undergone posterior aortopexy for compression of the left PV between the LA and the DA. The median age and weight of the patients at the time of operation were 5.5 months (range, 1-131 months) and 5.2 kg (range, 4.2-29.5 kg), respectively. The left PV obstruction was initially diagnosed on echocardiography in four patients and computed tomography angiography in one patient. The median peak pressure gradient across the obstructed left PV was 7.3 mmHg (range, 4-20 mmHg). Concomitant procedures were ventricular septal defect closure in one patient and patent ductus arteriosus ligation in one patient. Results: There was no PV obstruction on echocardiography in any of the patients after the operation except in the case of one patient who had diffuse pulmonary vein stenosis. The median follow-up duration was 34 months (range, 14-89 months), and during follow-up no incidence of the left PV obstruction was observed in any of the surviving patients. Conclusions: The posterior aortopexy technique could be a good surgical option for the left PV obstruction caused by compression between the LA and the anteriorly positioned DA.

We used a suture-and-open technique with a biatrial incision for primary sutureless repair of total anomalous pulmonary venous connection (TAPVC). With this technique, the common pulmonary venous sinus and its branching pulmonary veins are opened after completion of suturing of the left atrial incision to the pericardium around the common pulmonary venous sinus and its branching veins. The technique allows the primary sutureless repair of TAPVC to be done in a less bloody field under full-flow cardiopulmonary bypass. We have performed this technique in our recent 5 consecutive TAPVC patients without significant complications.

Background and Objectives We investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens. Methods Between March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire. Results Subjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p

We report a case of successful repair of truncus arteriosus (TA) associated with complete atrioventricular septal defect (c-AVSD) using a staged approach. TA associated with c-AVSD is a very rare congenital cardiac anomaly. No report of successful staged repair in South Korea has yet been published. We performed bilateral pulmonary artery banding when the patient was 33 days old, and total correction using an extracardiac conduit was performed at the age of 18 months. The patient recovered uneventfully and is doing well.

Background and objectives Patients with Kawasaki disease (KD) are clinically heterogeneous because its diagnosis is based solely on clinical observation and there are no definitive biomarkers. We dissected the clinical heterogeneity of KD patients using the KD-associated genetic variants. Methods We performed a genetic association analysis in several KD subgroups categorized by clinical characteristics using the KD-associated variants of the B lymphoid tyrosine kinase (BLK; rs6993775) and Fc gamma receptor II a (FCGR2A; rs1801274) in a large number of case (n=1,011) and control (n=4,533) samples. Results BLK and FCGR2A were very significantly associated with KD in Korean KD patients (odds ratio [OR],1.48; p=4.63×10⁻¹¹ for BLK, and OR, 1.26; p=1.42×10⁻⁴ for FCGR2A). However, in KD subgroup analysis, we found that neither BLK nor FCGR2A were associated with either incomplete Kawasaki disease (iKD) type patients or those older than 5 years of age (p>0.2), suggesting that patients with iKD or those older than 5 years of age are a unique subgroup of KD. In genetic association analysis after excluding iKD patients and those older than 5 years old, we found that BLK was associated with all KD subgroups, whereas FCGR2A was specifically associated with male KD patients younger than 1 year of age (OR, 2.22; p=2.35×10⁻⁵). Conclusions KD is a clinically and genetically heterogeneous disease. These findings will provide new insights into the clinical and genetic heterogeneity of KD.

Objectives We adopted an operative technique of pulmonary valve (PV) annular enlargement with valve repair in tetralogy of Fallot (TOF) correction to reduce postoperative pulmonary regurgitation (PR) 16 years ago. Here, we have evaluated the long-term results. Methods Between April 2000 and August 2005, 43 patients (26 men) with tetralogy of Fallot with pulmonary stenosis underwent PV annular enlargement with valve repair. The median age and body weight at the time of surgery were 14 months and 10.2 kg, respectively. Results There was no operative mortality. Mean postoperative PR grade at discharge was 0.93 ± 0.40 (none or trivial in 10 patients, mild in 27 patients, mild to moderate in 5 patients and moderate in 1 patient), and the mean postoperative pressure gradient across PV was 13.0 ± 10.9 mmHg. The mean follow-up duration was 131.9 ± 42.9 months. During follow-up, 1 reoperation was performed for residual ventricular septal defect. The mean PR grade at the last follow-up echocardiography was 1.59 ± 0.60 (mild in 17 patients, mild to moderate in 8 patients, moderate in 14 patients, moderate to severe in 1 patient and severe in 3 patients), and the mean pressure gradient was 22.7 ± 9.9 mmHg. We have compared the incidence of moderate or more PR with the incidence of patients who underwent simple transannular patch enlargement through propensity score matching. The PV repair group had a lower incidence of moderate or more PR compared with the simple transannular patch group (40% vs 68%, P = 0.04). Conclusions PV annular enlargement with valve repair has reasonable long-term results and yields a lower long-term incidence of significant PR compared with the simple transannular patch enlargement technique.

Introduction Despite recent advances in neonatal intensive care in Korea, few studies exist on the end-of-life decisions in newborns. In this study, we sought to examine the status of end-of-life decisions in neonates, changes over time, and affecting factors. Methods This is a retrospective study of neonates who died between 2001 and 2015 in the neonatal intensive care unit of Dong-A University Hospital in Busan. The types of end-of-life decisions were divided into active resuscitation, withholding treatment, and withdrawing treatment. The study period was divided into 3 time frames using 5-year intervals to investigate changes over time. To identify the associated factors, we analyzed the demographic and clinical characteristics of the neonates and their parents using the χ2 test and independent t test. Results Of the neonatal deaths included in the analysis (n = 222), active resuscitation, withholding treatment, and withdrawing treatment groups accounted for 73.4%, 25.2%, and 1.4% of cases, respectively. When comparing changes over time, between period 1 (2001-2005), 2 (2006-2010), and 3 (2011-2015), the proportion of active resuscitation decreased significantly, from 80.9% to 60.8%, while that of nonactive resuscitation increased significantly from 19.1% to 39.2%. The factors associated with end-of-life decisions were the clinical condition of the neonate at the time of death, rather than general characteristics or socioeconomic factors. Conclusions In Korea, changes in the decisions on end-of-life care in neonates are shifting from active resuscitation to nonactive resuscitation based on clinical conditions.

Background As syndromic short stature and overgrowth are heterogeneous and the list of causative genes is rapidly expanding, there is an unmet need for identifying genetic causes based on conventional gene testing or karyotyping. Early diagnosis leads to the proper management of the patient and providing genetic counseling for family members at risk in a timely manner. Materials and methods We conducted targeted exome sequencing to identify the genetic causes of undiagnosed syndromic short stature or overgrowth in 15 pediatric patients from 13 families in Korea. We applied targeted exome sequencing using the Next Seq platform and a TruSight One panel. Results Among the 13 families, 6 different disorders in 8 patients with short stature or overgrowth were identified, and the diagnostic yield was 46.2%. One boy with overgrowth had a TGFB3 gene mutation. In the short stature group, Coffin-Lowry syndrome (CLS), trichorhinophalangeal syndrome, DYRK1A haploinsufficiency syndrome, short stature with optic atrophy and Pelger-Huet anomaly syndrome with recurrent hepatitis, and type 4 Meier-Gorlin syndrome were identified. One CLS patient had a co-existing monogenic disease, congenital glaucoma, caused by the compound heterozygote mutations of the CYP1B1 gene. Conclusion Targeted exome sequencing is a powerful method for diagnosing syndromic growth disorders. It enables us to understand molecular pathophysiology and investigate new treatments for growth disorders.

The presence of fragmented QRS is a known marker of heterogeneous ventricular activation around the myocardial scar area. We validated whether fragmented QRS shows any association with ventricular dysfunction, fibrosis, or ventricular arrhythmias in patients with Duchenne muscular dystrophy (DMD). Thirty-seven patients with DMD were evaluated using electrocardiography (ECG), echocardiography, cardiac magnetic resonance, and 24-h Holter monitoring. Associations between fragmented QRS and ventricular dysfunction, fibrosis, or ventricular arrhythmia were investigated. Fragmented QRS complexes were present in 31 of 37 (83.7%) patients, and they were associated with a significantly lower left ventricular ejection fraction along with an increased left ventricular Tei index as evaluated by echocardiography, and more frequent ventricular arrhythmia as indicated using 24-hour Holter monitoring compared with patients without fragmented QRS. The number of leads with fragmented QRS correlated negatively with left ventricular ejection fraction both using echocardiography (r = -0.616) and CMR (r = -0.516). Further, the number of leads with fragmented QRS showed a significant correlation with several other echocardiographic measurements (mitral Em and Sm, and left ventricular Tei index), and 2-dimensional speckle-tracking echocardiography derived global left ventricular longitudinal strain. The frequency of ventricular arrhythmia observed using Holter monitoring showed a significant positive correlation with the frequency of fragmented QRS on ECG (r = 0.674). There was a positive trend of correlation between fragmented QRS and the amount of myocardial fibrosis as assessed by late gadolinium enhancement using CMR, but the statistical significance of the relationship was low (r = 0.433, p = 0.056). Fragmentation of QRS complexes is associated with degrees of left ventricular dysfunction, fibrosis, and ventricular arrhythmias in patients with DMD.

Uhl’s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl’s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.

Background and Objectives Kawasaki disease (KD) is an acute systemic vasculitis that affects the coronary arteries. Abnormal immune reactions are thought to contribute to disease pathogenesis. The effect of immunoglobulin (Ig) isotype (IgG, IgA, IgM, and IgE) on inflammatory data and clinical outcomes of patients with KD was examined. Methods Ig levels in 241 patients with KD were measured during the acute, subacute, convalescent, and normal phases of the disease. Results Compared with reference Ig values, IgG, IgA, and IgM levels were significantly higher in the subacute phase, while IgE levels were elevated in 73.9% (178/241) of patients with KD in all clinical phases. However, high IgE levels were not associated with clinical outcomes, including intravenous immunoglobulin unresponsiveness and coronary artery lesions (CALs). Significantly more CALs were observed in the high IgA group than in the normal IgA group (44.7% vs. 20.8%, respectively; p

Objectives We investigated the effect of balloon pulmonary valvuloplasty (BPV) as a palliative procedure for patients with tetralogy of Fallot (TOF). Methods This was a retrospective single-center study conducted between 2008 and 2014. We classified patients into three groups according to palliation: treatment with BPV (group I), with a Blalock–Taussig shunt (group II), and with infundibulectomy (group III). The growth of the pulmonary valve (PV) annulus and need for transannular patching (TAP) during total correction were compared between groups. Results Forty-eight patients were enrolled: 31, 10, and 7 in groups I, II, and III, respectively. The mean ages at palliation were 26.3 ± 23.6, 21.5 ± 16.4, and 15.2 ± 4.2 days in groups I, II, and III, respectively (P = .867). The success rate of BPV was 90.3%. The initial PV z-score before palliation did not differ between groups I, II, and III (−3.14 ± 1.44, −3.84 ± 1.47, and −3.71 ± 1.68; P = .494); the preoperative PV z-score before total correction was larger in group I than in group II (−1.19 ± 1.14 and −3.05 ± 1.19; P = .001), and also larger in group III than in group II (group III: −1.10 ± 0.90; P = .003). The change in pulse oxygen saturation differed significantly between the three groups (P = .031), particularly between groups I and II (P = .010), and the change in PV z-score was greater in groups I and III than in group II (P

Pseudoaneurysm in the left ventricular outflow tract is a rare complication occurring after cardiac surgery. We report on the successful percutaneous device closure of a pseudoaneurysm in the left ventricular outflow tract, which developed in a 23-month-old girl after resection of a subaortic ridge. The pseudoaneurysm was closed using an Amplatzer Vascular Plug II.

A patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries without an intrapericardial pulmonary artery (PA) underwent a one-stage total correction, including both branch PA reconstructions, with a bovine pericardial roll at the age of 42 months. She was readmitted 54 months after the operation because of extracardiac conduit bacterial endocarditis and pulmonary infarction. The bovine pericardial roll between the right and left PAs was enucleated, and the surrounding fibrotic tissue (peel) was used as a new branch PA vascular conduit. A cardiac catheterization performed 86 months after the peel operation showed no aneurysmal dilatation, stenosis, or thrombotic occlusion.

Background Conduit survival without significant dysfunction is important when selecting the right ventricular outflow tract conduit. We made an expanded polytetrafluoroethylene tricuspid valved conduit using a simplified technique. We aimed to investigate the midterm functional results and longevity of this conduit. Methods Between November 2008 and December 2016, four hospitals in Korea implanted 145 valved conduits. We retrospectively analyzed their functional results and longevity. Results The patients' median age at operation was 36.6 months; the median body weight was 11.3 kg. The mean follow-up duration was 32.3 ± 24.5 months. There were four inhospital deaths and three late deaths, but there were no conduit-related deaths. The mean peak systolic pressure gradient across the conduit was 14.7 ± 8.3 mm Hg and 31.6 ± 17.7 mm Hg at discharge and last follow-up, respectively. Six patients (4.4%) had moderate or more conduit valve regurgitation at last follow-up. Conduit dysfunction was observed in 30 patients (21.9%), mainly caused by increased pressure gradient (24 of 30, 80%). Freedom from conduit dysfunction was 88.1% and 58.5% at 3 and 5 years, respectively. Lower freedom from conduit dysfunction was observed in small conduits. Eleven patients (7.8%) underwent conduit explantation, and freedom from explantation was 94.8% and 81.7% at 3 and 5 years, respectively. The main cause of explantation was conduit stenosis. Small conduits tended to have lower freedom from explantation. Conclusions Functional results and longevity of our expanded polytetrafluoroethylene tricuspid valved conduit are acceptable. Although our conduits tend to have increasing pressure gradient over time, especially in small conduits, they have low incidence of moderate or more regurgitation.

Kawasaki disease (KD) is a self-limited systemic vasculitis of an unknown pathogenesis mainly affecting children

Background and aim to read We report the results of a bicuspid expanded polytetrafluoroethylene (ePTFE) valved conduit used for right ventricular outflow tract reconstruction (RVOTR). Methods Between November 2005 and February 2009, 12 conduits were used for RVOTR. The mean age and weight of patients were 43.5 ± 46.4 months and 13.4 ± 8.6 kg. The main diagnosis was tetralogy of Fallot with pulmonary atresia in eight patients. The most common conduit size was 18 mm. The mean follow-up was 88.0 ± 35.9 months. Results There were no operative and late mortalities. At discharge, the mean peak systolic pressure gradient across the RVOT was 14.1 ± 11.3 mmHg. There was no conduit valve regurgitation in nine patients. At the latest echocardiography (mean follow-up: 84.3 ± 35.5 months), the mean peak systolic pressure gradient across the RVOT was 59.7 ± 20.2 mmHg, and there was no conduit valve regurgitation in six patients. Freedom from conduit malfunction was 100% and 83.3%, at 1 and 8 years, respectively. Two conduits were explanted due to sternal compression and four from conduit malfunction. Freedom from explantation was 83.3% and 74.2% at 2 and 8 years, respectively. Conclusions ePTFE bicuspid valved conduit has good late function in terms of valve regurgitation, but the pressure gradient across the conduit increases with time, which is the main cause of conduit failure and explantation.

Objectives Postoperative left pulmonary artery (LPA) kinking is problematic in repair of tetralogy of Fallot (TOF). We used angioplasty of the proximal LPA with an anterior wall flap of the main pulmonary artery (MPA) to prevent this problem. Methods We have used an anterior wall of the MPA as a flap for LPA angioplasty in 42 patients with TOF and pulmonary stenosis (26 males and 16 females) since February 2007. During the same period, 116 patients underwent total repair of TOF with pulmonary stenosis. The median age was 7.1 months (range, 3-88.8), and the median weight was 8.4 kg (range, 5-27). The indications for LPA angioplasty were acute-angle proximal LPA in 17 (40.5%), proximal LPA stenosis in 6 (14.3%), acute-angle and stenotic proximal LPA in 18 (43%), and short and small distal MPA in 1 patient. The patches used for MPA and LPA reconstruction were glutaraldehyde-treated autologous pericardium in 34 and bovine pericardium in 8 patients. Results No operative or late death occurred. The mean follow-up duration after surgery was 26.4 ± 18.6 months (range, 0.5-67). During the follow-up period, reoperation for LPA stenosis or kinking was not required in any patient; however, balloon angioplasty was performed with good results in 4 patients (9.5%). No postoperative kinking of the proximal LPA occurred. Echocardiography or computed tomography angiography at the recent follow-up visit demonstrated good branch pulmonary arteries in all patients. Conclusions Angioplasty of the proximal LPA using an anterior wall flap of the MPA in the patients with TOF and pulmonary stenosis is an effective method in the management of acute angle and/or stenosis of LPA without postoperative kinking.

Background Airway compression caused by an enlarged right pulmonary artery (RPA) in patients with a large shunt can usually be managed with intracardiac repair and concomitant anterior aortopexy. However, anterior aortopexy can be less effective or even dangerous in patients with coexisting arch anomaly due to excessive tension at the arch repair site. We have adopted anterior translocation of RPA without aortic transection in the group of patients with a high risk of postoperative airway compression. We reviewed the early and midterm results of this technique. Methods From February 2006 to January 2013, 8 patients underwent RPA anterior translocation as a concomitant procedure in one-stage repair of ventricular septal defect (VSD) and aortic arch anomaly to avoid postoperative airway problems. The enlarged RPA was disconnected from the main pulmonary artery (MPA) at its origin and was relocated anterior to the ascending aorta, and subsequently reimplanted to the U-shaped trapdoor incision at the anterolateral MPA wall. The mean age at operation was 34 days (median, 14 days, 6 to 77 days), and the mean body weight was 3.6 kg (2.15 to 5.5 kg). All patients had coarctation of the aorta and VSD except 1 who had aortic arch interruption. Five patients were dependent on a ventilator preoperatively. Six patients had evidence of preoperative bronchial compression (left; 4, right and left; 2), and 2 had a high probability of postoperative bronchial compression due to unusual anterior location of the descending aorta. Results There was no early or late death. There were no postoperative airway problems such as reintubation or left lung atelectasis. Widely patent RPA was confirmed on postoperative computed tomographic angiography in all patients. The mean follow-up duration was 54.0 ± 17.1 months. One patient required balloon angioplasty for mild stenosis at the clamping site 3 years after the operation. All patients had no RPA stenosis at the latest follow-up evaluation. Conclusions Anterior translocation of the RPA as a concomitant procedure in one-stage repair of VSD and arch anomaly is a safe and effective procedure to avoid postoperative airway problems in high-risk patients.

The management of pulmonary atresia with a ventricular septal defect (PA/VSD) depends on the anatomy of the pulmonary artery or on the surgical strategy used at individual institutions. In our institution, we adopted a right ventricle-to-pulmonary artery (RV-PA) shunt in 2011 as a palliative procedure for PA/VSD to overcome the disadvantages of a Blalock-Taussig shunt. We evaluated the results of the RV-PA shunt as initial palliative surgery for PA/VSD. Thirteen patients with ductus-dependent PA/VSD from August 2011 to August 2015 were enrolled. The mean age at surgery was 17.9 ± 15.3 (range 5–60) days, and the mean body weight was 2.9 ± 0.6 (range 2.2–4.0) kg. A Gore-Tex tube graft was used in all patients. We retrospectively observed intra- and postoperative complications, early and late mortality, and palliation duration to definitive repair. Left pulmonary artery angioplasty was performed as a concomitant procedure in three patients. There were no early hospital mortalities, although two inter-stage deaths occurred 34 and 47 days postoperatively: one patient died of aspiration and the other of right ventricular outflow tract (RVOT) pseudoaneurysm rupture. Two patients (15.4%) required the extracorporeal membrane oxygenation support postoperatively: one because of failure to wean from a bypass caused by persistent hypoxemia and the other because of sudden massive bleeding from the RVOT suture line in the intensive care unit 2 days postoperatively. These two patients underwent second-stage definitive repair successfully. Five patients (41.7%) required catheter intervention, for juxtaductal left pulmonary artery stenosis in three patients, right pulmonary artery stenosis in one, and shunt inflow stenosis in one. Two patients (15.4%) required re-operation because of shunt inflow stenosis and RVOT pseudoaneurysm, respectively. All patients who survived the RV-PA shunt underwent total correction at a mean interval of 13.1 months. A RV-PA shunt is an option for the initial palliation of ductus-dependent PA/VSD. Major complications can occur, including RVOT pseudoaneurysm, shunt inflow stenosis, persistent hypoxemia during the immediate postoperative period, and dehiscence of the shunt anastomosis site. Caution should be taken when performing the RV-PA shunt for palliation of PA/VSD.

In this study, we sought predictors of mortality in children with acute myocarditis and of incomplete recovery in the survivor group. We classified our patients into three groups according to their outcomes at last follow-up: full recovery was classified as group I, incomplete recovery was classified as group II, and death was classified as group III. In total, 55 patients were enrolled in the study: 33 patients in group I, 11 patients in group II, and 11 patients in group III. The initial left ventricular fractional shortening – left ventricular fractional shortening – was significantly lower in group III (p=0.001), and the left ventricular end-diastolic dimension z score was higher in groups II and III compared with group I (p=0.000). A multivariate analysis showed that the left ventricular end-diastolic dimension z score (odds ratio (OR), 1.251; 95% confidence interval (CI), 1.004–1.559), extracorporeal membrane oxygenation (OR, 9.842; 95% CI, 1.044–92.764), and epinephrine infusion (OR, 18.552; 95% CI, 1.759–195.705) were significant predictors of mortality. The left ventricular end-diastolic dimension z score was the only factor that predicted incomplete recovery in the survivor group (OR, 1.360; 95% CI, 1.066–1.734; p=0.013). The receiver operating characteristic curve of the left ventricular end-diastolic dimension z score at admission showed a cut-off level of 3.01 for predicting mortality (95% CI, 0.714–0.948). In conclusion, a high left ventricular end-diastolic dimension z score on admission was a significant predictor of worse outcomes, both regarding mortality and incomplete recovery.

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7 days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6 g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34-5.68; P = 0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.

Background: The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery. Materials and Methods: From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was 13.4±10.2 days (4 to 39 days) and mean body weight was 3.48±0.33 ㎏ (2.88 to 3.88 ㎏). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion. Results: There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p＞0.1). There was no late death. The mean follow-up duration was 52.1±43.0 months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1. Conclusion: The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.

Iatrogenic pseudoaneurysms are extremely rare in children. Conventional management of pseudoaneurysms in adults has included surgical repair, ultrasound-guided compression, and more recently, endovascular embolization. However, in infants and children, there is little information regarding the applicability of such treatment modalities, which have been effective in adults, because of its rarity. Here, we present the case of a 6-month-old infant who developed a postprocedural pseudoaneurysm of the external iliac artery, which was successfully treated with ultrasound-guided percutaneous thrombin injection.

Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, 11.7±13.2 days) and weight at operation ranged from 2.2 to 4.8 ㎏ (mean, 3.17±0.52 ㎏). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group Ⅰ) and RMBTS in 8 (group Ⅱ). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean 26.0±22.8 months). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.

Rationale Infantile-onset hypertrophic cardiomyopathy (HCMP) should be considered a largely genetic condition, although its onset is most often triggered by infection. Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a rare autosomal recessive inborn error of mitochondrial fatty acid β-oxidation that often causes severe cardiomyopathy and/or sudden death during the neonatal period. Patient concerns Herein, we report an infant with VLCAD deficiency who presented with severe cardiac manifestations, including massive pericardial effusion and HCMP. The subject's older sister died of unknown causes at three days of age; however, the subject exhibited a normal tandem mass-spectrometry profile during the neonatal period. Diagnoses During her later cardiac presentation, the subject's C-14 and C-18 levels became elevated, and she was determined, via the conducted molecular analysis, to harbor a novel homozygous frameshift mutation (c.103_112dup) in ACADVL. Interventions After VLCAD deficiency diagnosis, the subject was treated with the administration of a medium chain triglyceride formula and fluid therapy. Outcomes The subject's cardiac status was markedly improved by the dietary intervention and fluid therapy. Lessons This report highlights that genetic mutations should be investigated as possible causes of infantile-onset HCMP, and that early diagnosis and intervention can prevent mortality for patients with VLCAD deficiency.

Background Reconstruction of branch pulmonary arteries (PAs) can be demanding in redo congenital cardiac surgeries. Sutureless patch angioplasty could be a useful method to solve this problem, and we evaluated the feasibility of sutureless patch angioplasty for postoperative PA stenosis in patients who underwent congenital cardiac surgery. Methods We retrospectively reviewed 28 patients (19 males and 9 females) who underwent sutureless patch angioplasty for postoperative PA stenosis between November 2004 and April 2015. The median age was 7.3 months (range, 4.3 to 54.7), and the median weight was 7.2 kg (range, 5.3 to 12.2 kg). Right PA angioplasty was performed in 5, left PA angioplasty in 10, and both sides in 13 patients. The most common original diagnosis was hypoplastic left heart syndrome (n = 18 of 28, 64.3%). Concomitant surgeries were the bidirectional cavopulmonary shunt in 24 patients, the Fontan operation in 3, and the Rastelli operation in 1 patient. Results No operative death occurred. However, 2 late deaths were recorded, and both were unrelated to PA angioplasty. Mean follow-up duration was 60.9 ± 33.1 months. None of the patients had postoperative bleeding or thrombotic occlusion. No reoperation for PA restenosis was performed, and only 1 patient (3.6%) had a PA balloon angioplasty with a good result 12.9 months after the operation. Echocardiography or computed tomography angiography at the recent follow-up showed good branch PAs in all patients. Conclusions Sutureless patch angioplasty for postoperative PA stenosis could simplify PA angioplasty, and be a safe and effective method for PA reconstruction in patients who undergo congenital cardiac surgery.

Multislice CT (MSCT) allows high-quality volume-rendered (VR) and composite volume-rendered images.To investigate the clinical usefulness of composite VR images in the evaluation of the relationship between cardiovascular structures and the airway in children with complex congenital heart disease (CHD).Four- or 16-slice MSCT scanning was performed consecutively in 77 children (mean age 6.4 months) with CHD and respiratory symptoms, a chest radiographic abnormality, or abnormal course of the pulmonary artery on ECHO. MSCT scanning was performed during breathing or after sedation. Contrast medium (2 ml/kg) was administered through a pedal venous route or arm vein in all patients. The VR technique was used to reconstruct the cardiovascular structures and airway, and then both VR images were composed using the commercial software (VoxelPlus(2); Daejeon, Korea).Stenoses were seen in the trachea in 1 patient and in the bronchi in 14 patients (19%). Other patients with complex CHD did not have significant airway stenoses.Composite VR images with MSCT can provide more exact airway images in relationship to the surrounding cardiovascular structures and thus help in optimizing management strategies in treating CHD.

Background Accurate coronary reimplantation is the most important component in the arterial switch operation. It is especially demanding for the less experienced surgeons. We compared the result of the technique of coronary reimplantation after neoaortic reconstruction with that of the open trap door technique. Methods From March 1994 to June 2004, 103 consecutive patients underwent the arterial switch operation by one surgeon. Patients who underwent coronary artery transfer with other modified techniques were excluded. Diagnoses of 94 patients were transposition of the great arteries with intact ventricular septum (n = 50), transposition of the great arteries with ventricular septal defect (n = 26), and the Taussig-Bing anomaly (n = 18). An aortic arch anomaly was present in 13 patients. The median age of the patients was 12 days and the mean body weight was 3.5 kg. Coronary reimplantation after neoaortic reconstruction was applied to 34 patients (group I), and the open trap door technique was applied to the rest (group II). Results Preoperative data were similar in both groups. Four patients in group II required intraoperative revision of a transferred coronary artery, and 1 patient with an intramural left coronary artery in group I had a conversion to free grafting using the left subclavian artery. Overall early mortality was 17.0% (16 of 94). Mortality in group I (1 of 34; 2.9%) was significantly lower than in group II (15 of 60; 25.0%) ( p = 0.008). The leading cause of death in group II was low cardiac output (n = 9). During the follow-up, an aortic regurgitation of greater than mild was detected in 2 patients in group II. Conclusions Coronary reimplantation after neoaoartic reconstruction is an attractive method to minimize coronary artery transfer-related mortality or morbidity.

Background The reimplantation of the coronary arteries from a single aortic sinus (single sinus coronary artery) in an arterial switch operation remains a technically challenging procedure. The technique of coronary transfer in this situation should be individualized depending on coronary ostial anatomy. We reviewed our techniques of coronary reimplantation with early and midterm results. Methods Among 103 patients who underwent arterial switch operations from March 1994 to June 2004, 16 (15.5%) had single sinus coronary artery (median age, 9 days; mean body weight, 3.5 kg). Fourteen patients (14/16, 87.5%) had coronary arteries from right facing sinus (sinus 2). Of these 14 patients, 11 had separate ostia including intramural course of the left coronary artery (n = 9) and 3 had single ostium. Two patients (2/16, 12.5%) had coronary arteries from left facing sinus (sinus 1) with single ostium. Aortic arch obstruction was associated in 5 patients. Results All 5 single sinus coronary arteries with single ostium were reimplanted with the trap-door technique. Of the 11 patients with separate ostia, 8 underwent coronary transfer with the aortocoronary flap technique and 3 with the double-button technique. Two of the 3 patients who underwent the double-button technique required left coronary artery bypass using left subclavian artery free graft as the salvaging procedure. There was one early death (1/16, 6.3%), which occurred during our earlier experience, in a patient who had arch anomaly and intramural left coronary artery. There was no late death. All but one patient had good ventricular function. Conclusions All single sinus coronary artery with single ostium can be transferred with the trap-door technique with excellent results. The aortocoronary flap technique in those with separate ostia with or without intramural left coronary artery may be a good option. However, reimplantation of the intramural left coronary artery using separate coronary buttons should be performed with great care.

Airway compression by dilated right pulmonary artery (RPA) in infants with congenital heart disease can cause severe respiratory insufficiency and increase postoperative morbidity. Anterior aortopexy can be a good solution. However, in a case in which aortic arch repair is required, anterior aortopexy might not be effective or can be dangerous. Anterior translocation of the RPA can be a good option to avoid postoperative airway obstruction. We adopted this technique in a one-stage complete repair of distal aortopulmonary window, aortic origin of the RPA, interrupted aortic arch, and ventricular septal defect in a neonate with compression of both main bronchi. After 37 months of follow-up, no problem was encountered with her airway or RPA. We describe the technique used and the results obtained.

Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p < 1 × 10(-5)). Of these, only 2 SNPs (rs12068753 and rs4786091) demonstrated a significant association with the CRP level in replication study of 473 KD patients (p < 0.05). The SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10(-6) according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10(-8) according to the replication study; beta = 3.97 and p = 1.11 × 10(-13) according to combined analysis) and explained 8.1% of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1% of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.

Purpose The aims of this study were to document our single-center experience with pediatric acute fulminant myocarditis (AFM) and to investigate its clinical features and short-term outcomes. Methods We performed a retrospective chart review of all children 24 hours after admission in 4 of the 13 patients (30.7%), and 3 of those 4 patients (75%) died. Conclusion AFM outcomes may be associated with complete atrioventricular block upon hospital admission, left ventricular fractional shortening at admission, time from admission to the initiation of ECMO support, initial brain natriuretic peptide level, and peak blood urea nitrogen level.

OBJECTIVES: This study was undertaken to assess the early and long-term results of total repair of tetralogy of Fallot (TOF) and to identify the risk factors associated with late right ventricular (RV) dilatation. METHODS: The medical records of 326 patients (male:female = 192:134) who underwent total repair of TOF at Pusan National and Dong-A University Hospitals between July 1991 and May 2011 were retrospectively reviewed. Median age and weight at the time of operation were 13.0 months and 8.7 kg, respectively. Right ventricular end-diastolic dimensions and left ventricular end-diastolic dimensions were obtained during follow-up echocardiography to identify the risk factors associated with late RV dilatation. RESULTS: There were one operative death (0.3%) and 8 late deaths (2.5%). Of late deaths, two were related to operation-related cardiac problems. Overall survival rates at 5, 10, and 15 years were 97.0%, 95.4%, and 95.4%, and the corresponding freedom from cardiac death were 98.8%, 98.8%, and 98.8%, respectively. Freedom from re-operation and re-intervention were 84.4%, 74.2% and 74.2%. Six patients underwent pulmonary valve replacement during the follow-up period. Transannular patch (P= 0.036) and postoperative ventilator support period (P< 0.001) were found to be significant risk factors of late RV dilatation in multivariate analysis. CONCLUSIONS: Total correction of TOF can be performed with a very low mortality rate. However, the postoperative re-operation or re-intervention rates remain relatively high. Late RV dilatation after total repair of TOF was found to be associated with transannular patch enlargement and a longer postoperative ventilator support period.

We report on an infant presenting with acute cardiogenic shock. She was eventually diagnosed with pseudohypoparathyroidism, which is a heterogeneous group of disorders characterized by severe hypocalcemia, hyperphosphatemia, and increased parathyroid hormone. The patient responded dramatically to calcium and vitamin D supplementation; left ventricular systolic function was normalized within days of treatment. Although the diagnosis of pseudohypoparathyroidism is rare, this case is a reminder of the importance of obtaining calcium levels in patients presenting in acute cardiogenic shock.

Objectives We report a single institute experience of transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by a systemic-pulmonary shunt in patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). In addition, we compare patients with or without an additional source of flow to support the pulmonary circulation after successful pulmonary valvotomy. Methods All neonates with PAIVS or CPS who underwent primary transcatheter pulmonary valvotomy between January 2004 and December 2010 were reviewed retrospectively. Some of them needed an additional source of flow to support the pulmonary circulation. We performed a comparison between those who required an additional source of pulmonary flow and those who did not. Results The initial procedure was successful in 20 out of 22 patients (seven of nine with PAIVS; all of 13 with CPS), but 10 of them needed an additional source of flow to support the pulmonary circulation: nine had arterial duct stenting and one had surgical Blalock-Taussig shunt. There were no deaths or major acute complications, except for femoral artery occlusion in three patients. The bipartite right ventricular morphology, the tricuspid z-score of ≤−0.74, the tricuspid to mitral valve ratio of ≤0.9, and the z-score of the diastolic interventricular septal thickness ≥2.37 in preprocedural examination showed more tendency of needing shunt placement. Conclusion Transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by the arterial duct stent implantation was an effective approach in those patients. The angiographic distinction between CPS and PAIVS did not affect anything in this study including the procedural method, success, and odds for reintervention. The degree of right ventricle cavity hypoplasia provided the main restriction to forward flow after pulmonary valvotomy.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to the Children's Hospital of Pusan National University because of persistent fever, rash, abdominal distension, generalized edema, lymphadenopathy, and eosinophilia. He previously received vancomycin and ceftriaxone for 10 days at another hospital. He developed acute kidney injury with nephrotic range proteinuria and hypocomplementemia. A subsequent renal biopsy indicated the presence of acute tubular necrosis (ATN) and late exudative phase of postinfectious glomerulonephritis (PIGN). Systemic symptoms and renal function improved with corticosteroid therapy after the discontinuation of vancomycin. Here, we describe a biopsy-proven case of severe ATN that manifested as a part of vancomycin-induced DRESS syndrome with coincident PIGN. It is important for clinicians to be aware of this syndrome due to its severity and potentially fatal nature.