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Background: The percutaneous transcatheter closure of patent ductus arteriosus (PDA) has been a widely used treatment method. However, PDA device closure in neonates or patients with specific PDA morphology has been difficult due to the protrusion of the device into the descending aorta. The right angle between the disc and plug causes some degree of protrusion of the disc into the descending aorta because normal PDA forms an acute angle with the descending aorta. Objectives: There have been limited data about the angles of PDA since Mancini's study in 1951, and new studies are required in this regard. This study measured the angles between PDA and descending aorta through angiography in a beating heart. Methods: Within December 2008 to November 2016, 190 patients undergoing percutaneous PDA occlusion were included in this study. Retrospectively, the mean angle of PDA was measured by three cardiologists between the longitudinal axis of the descending aorta and the longitudinal axis of the PDA through an aortogram. The patients were divided into three groups according to age (group A: under 1, group B: 1-6, and group C: over 6 years of age) and PDA morphology based on Krichenko's classification (type A: conical PDA, type B: window PDA, type C: tubular PDA, type D: complex PDA, and type E: elongated PDA). Results: Of 190 study patients, 135 patients were female, and the median age of the patients was 7 years (range: 75 days to 60 years). The mean angle of PDA was 48.2±12.0°. There were no statistical differences regarding PDA angle among the groups classified by age and PDA morphology. [ABSTRACT FROM AUTHOR]

An important, albeit rare, cause of fetal bradycardia is long QT syndrome (LQTS). Congenital LQTS is an ion channelopathy caused by mutations in genes encoding cardiac ion channel proteins. Fetal onset of LQTS imposes high risk of life-threatening tachyarrhythmias and sudden cardiac death. Here, we report the case of a female newborn with fetal onset of bradycardia and a 2:1 atrioventricular (AV) block. After birth, a 12-lead electrocardiogram (ECG) revealed bradycardia with QT prolongation of a corrected QT (QTc) interval of 680 ms and pseudo 2:1 AV block. Genetic testing identified a heterozygous Gly402Ser (c.1204G>A) mutation in CACNA1C, confirming the diagnosis of LQTS type 8 (LQT8). The patient received propranolol at a daily dose of 2 mg/kg. Mexiletine was subsequently administered owing to the sustained prolongation of the QT interval and pseudo 2:1 AV block. One week after mexiletine inception, the ECG still showed QT interval prolongation (QTc, 632 ms), but no AV block was observed. There were no life-threatening tachyarrhythmias in a follow-up period of 13 months. [ABSTRACT FROM AUTHOR]

Background: Multislice CT (MSCT) allows high-quality volume-rendered (VR) and composite volume-rendered images. Objective: 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). Materials and methods: 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 (VoxelPlus²; Daejeon, Korea). Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Oesophageal varices due to total anomalous pulmonary venous connection (TAPVC) is very rare. Additionally, the infradiaphragmatic type is the most common type of oesophageal varices due to TAPVC. Paraoesophageal varices due to stenosis of the vertical vein of supracardiac TAPVC has not previously been reported. We describe paraoesophageal varices developed as a result of a connection between the left lower pulmonary vein and the umbilicovitelline venous system because of stenosis of the proximal vertical vein in supracardiac type TAPVC in a 3-day-old female newborn who presented with general cyanosis, tachypnoea and dyspnoea. [ABSTRACT FROM AUTHOR]

The growth potential of the neoaorta after the stage I Norwood procedure could be limited when the patient has a small ascending aorta. Patients with hypoplastic left heart syndrome usually have a large main pulmonary artery (MPA) and from its posterior wall both pulmonary arteries arise. We describe a modified technique that spares a large anterior wall of the MPA in the neoaortic reconstruction of Norwood procedure to enhance the growth potential of the neoaorta. [ABSTRACT FROM AUTHOR]

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 are from 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We present the case of a 5-year-old child with coronary complications due to Kawasaki disease; this patient unintentionally underwent both dual-source computed tomography (DSCT) coronary angiography and invasive coronary angiographic examination in 2 months. This case highlights the strong consistency of the results between DSCT coronary angiography and invasive coronary angiography. Compared to conventional invasive coronary angiography, DSCT coronary angiography offered additional advantages such as minimal invasiveness and less radiation exposure. [ABSTRACT FROM AUTHOR]

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 <18 years old who were diagnosed with AFM between October 2008 and February 2013. Data about patient demographics, initial symptoms, investigation results, management, and outcomes between survivors and nonsurvivors were collected. Results: Seventeen of 21 patients (80.9%) with myocarditis were diagnosed with AFM. Eleven patients (64.7%) survived to discharge, and 6 (35.3%) died. Electrocardiography on admission revealed dysrhythmia in 10 patients (58.8%); of these, all 7 patients with a complete atrioventricular block survived. Fractional shortening upon admission was significantly different between the survivors (16%) and nonsurvivors (8.5%) (P =0.01). Of the serial biochemical markers, only the initial brain natriuretic peptide (P =0.03) and peak blood urea nitrogen levels (P =0.02) were significantly different. Of 17 patients, 4 (23.5%) required medical treatment only. Extracorporeal membrane oxygenation (ECMO) was performed in 13 patients (76.5%); the survival rate in these patients was 53.8%. ECMO support was initiated >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. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is an acute self-limited vasculitis of infants and children that manifests as fever and signs of mucocutaneous inflammation. Coronary artery aneurysms develop in approximately 15-25% of untreated children. Although the etiology of KD is largely unknown, epidemiologic data suggest the importance of genetic factors in the susceptibility to KD. In order to identify genetic variants that influence KD susceptibility, we performed a genome-wide association study (GWAS) using Affymetrix SNP array 6.0 in 186 Korean KD patients and 600 healthy controls; 18 and 26 genomic regions with one or more sequence variants were associated with KD and KD with coronary artery lesions (CALs), respectively ( p < 1 × 10). Of these, one locus on chromosome 1p31 (rs527409) was replicated in 266 children with KD and 600 normal controls (odds ratio [OR] = 2.90, 95% confidence interval [CI] = 1.85-4.54, P = 1.46 × 10); and a PELI1 locus on chromosome 2p13.3 (rs7604693) was replicated in 86 KD patients with CALs and 600 controls (OR = 2.70, 95% CI = 1.77-4.12, P = 2.00 × 10). These results implicate a locus in the 1p31 region and the PELI1 gene locus in the 2p13.3 region as susceptibility loci for KD and CALs, respectively. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]