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Objectives: To evaluate early- and long-term outcomes of the surgical treatment for coarctation of the aorta based on a new classification system. Methods: A retrospective clinical review of 111 patients with coarctation of the aorta who underwent surgery (March 2011 to August 2020) was performed. We categorised coarctation of the aorta into type I, with all three head vessels tightly packed; type II, with the left subclavian artery separated from the two other head vessels; and type III, with all three head vessels separated from one another. Each type included subtype a, with a short isthmic portion, and subtype b, with a long isthmic portion. Results: The median patient age and weight at operation were 8 (range, 1–1490) days and 3.2 (range, 1.9–18.5) kg, respectively. Extended end-to-end anastomosis was performed via sternotomy in 54, via thoracotomy in 12, end-to-side anastomosis in 31, autologous main pulmonary artery patch augmentation in 12, and modified end-to-end anastomosis combined with subclavian artery flap aortoplasty in two patients. There was one (0.9%) case of early mortality and 12 (10.8%) cases of post-operative complications. Two (1.8%) late deaths occurred during follow-up. Five (4.5%) patients underwent balloon dilatation and three (2.7%) underwent reoperation for restenosis of coarctation of the aorta. All patients with type Ia (21 patients, 18.9%) underwent extended end-to-end anastomosis via sternotomy or thoracotomy. Conclusions: According to the early and late outcomes observed in this study, surgical treatment of coarctation of the aorta using the new classification system could be safe and low risk.

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.

Background Myocarditis refers to the inflammation of the myocardium caused by infection or autoimmune disease that may or may not present with clinical manifestations, such as gastrointestinal symptoms, dyspnea, chest pain, or sudden death. Although myocarditis and coronary artery vasospasm may mimic ST-segment elevation myocardial infarction (STEMI) with normal coronary arteries on angiography, acute myocarditis rarely causes coronary artery spasm. Here, we report a case of coronary artery spasm with reversible electrocardiographic changes mimicking STEMI in an adolescent with acute myocarditis. Case presentation A 15-year-old boy present with sudden-onset repeated chest pain following a 3-day history of flu-like illness. Cardiac biomarkers were significantly elevated. Electrocardiography showed ST-segment elevation in the absence of detectable vasospasm on coronary angiography. These findings were consistent with the diagnosis of coronary artery spasm secondary to acute myocarditis. Treatment with immunoglobulin for 2 days improved his condition. The patient was discharged on the 12th day with complete resolution of symptoms and normalization of electrocardiogram findings. Conclusions We reported a case of coronary artery spasm due to acute myocarditis. This study highlights the importance of considering coronary artery spasm due to acute myocarditis as a differential diagnosis in patients presenting with signs of STEMI as these diseases have different medical management strategies.

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

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.

Kawasaki disease (KD) is an acute pediatric vasculitis that affects genetically susceptible infants and children. To identify coding variants that influence susceptibility to KD, we conducted whole exome sequencing of 159 patients with KD and 902 controls, and performed a replication study in an independent 586 cases and 732 controls. We identified five rare coding variants in five genes (FCRLA, PTGER4, IL17F, CARD11, and SIGLEC10) associated with KD (odds ratio [OR], 1.18–4.41; p = 0.0027–0.031). We also performed association analysis in 26 KD patients with coronary artery aneurysms (CAAs; diameter > 5 mm) and 124 patients without CAAs (diameter < 3 mm), and identified another five rare coding variants in five genes (FGFR4, IL31RA, FNDC1, MMP8, and FOXN1), which may be associated with CAA (OR, 3.89–37.3; p = 0.0058–0.0261). These results provide insights into new candidate genes and genetic variants potentially involved in the development of KD and CAA.

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.

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

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.

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