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

Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.
Arteriovenous Fistula ; Coronary Vessel Anomalies ; Coronary Vessels ; Echocardiography ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Pregnancy ; Prenatal Diagnosis

PURPOSE: The purpose of this study was to investigate the clinical significance of Bacille Calmette-Guérin (BCG) site reaction in terms of diagnosis and outcome prediction in young children with Kawasaki disease (KD). METHODS: The incidence of BCG site reaction in the respective age ranges was investigated in 1,058 patients who were admitted at Asan Medical Center between January 2006 and February 2017. The 416 patients under 18 months of age were enrolled as subjects for the analysis of the association between BCG site reaction and other laboratory and clinical findings. The analysis was performed separately in complete and incomplete KD groups. RESULTS: The incidence rate of BCG site reaction was peaked at 6–12 months (83%) and decreased with increasing age after 12 months in 1,058 patients (P < 0.001). The incidence rate was above 70% in KD aged less than 18 months and more frequent than those of cervical lymphadenopathy. The logistic regression analyses showed that the principal clinical findings including conjunctivitis (P=0.781), red lips/oral mucosa (P=0.963), rash (P=0.510), cervical lymphadenopathy (P=0.363), changes in extremities (P=0.283) and the coronary artery aneurysm (P=0.776) were not associated with the BCG site reaction. CONCLUSIONS: The BCG site reaction could be a useful diagnostic tool independent to principal clinical findings in KD developing in children aged < 18 months, who underwent BCG vaccination. Outcome of KD patients was not different between groups with or without the BCG site reaction in both complete KD and incomplete KD.
Aneurysm ; BCG Vaccine ; Child ; Chungcheongnam-do ; Conjunctivitis ; Coronary Vessels ; Diagnosis ; Erythema ; Exanthema ; Extremities ; Humans ; Incidence ; Logistic Models ; Lymphatic Diseases ; Mucocutaneous Lymph Node Syndrome ; Mucous Membrane ; Mycobacterium bovis ; Vaccination

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

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

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
Adolescent ; Arteriovenous Malformations* ; Fontan Procedure ; Heart Defects, Congenital ; Humans ; Male ; Vascular Diseases

To identify the Helicobacter pylori antigens operating during early infection in sera from infected infants using proteomics and immunoblot analysis. Two-dimensional (2D) large and small gel electrophoresis was performed using H. pylori strain 51. We performed 2D immunoglobulin G (IgG), immunoglobulin A (IgA), and immunoglobulin M (IgM) antibody immunoblotting using small gels on sera collected at the Gyeongsang National University Hospital from 4–11-month-old infants confirmed with H. pylori infection by pre-embedding immunoelectron microscopy. Immunoblot spots appearing to represent early infection markers in infant sera were compared to those of the large 2D gel for H. pylori strain 51. Corresponding spots were analyzed by matrix-assisted laser desorption/ionization time of flight-mass spectrometry (MALDI-TOF-MS). The peptide fingerprints obtained were searched in the National Center for Biotechnology Information (NCBI) database. Eight infant patients were confirmed with H. pylori infection based on urease tests, histopathologic examinations, and pre-embedding immunoelectron microscopy. One infant showed a 2D IgM immunoblot pattern that seemed to represent early infection. Immunoblot spots were compared with those from whole-cell extracts of H. pylori strain 51 and 18 spots were excised, digested in gel, and analyzed by MALDI-TOF-MS. Of the 10 peptide fingerprints obtained, the H. pylori proteins flagellin A (FlaA), urease β subunit (UreB), pyruvate ferredoxin oxidoreductase (POR), and translation elongation factor Ts (EF-Ts) were identified and appeared to be active during the early infection periods. These results might aid identification of serological markers for the serodiagnosis of early H. pylori infection in infants.
Biotechnology ; Electrophoresis ; Flagellin ; Gels ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoblotting ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Infant* ; Microscopy, Immunoelectron ; Peptide Elongation Factors ; Peptide Mapping ; Proteomics ; Pyruvate Synthase ; Serologic Tests ; Spectrum Analysis ; Urease

PURPOSE: This study investigated predictors of unresponsiveness to second-line intravenous immunoglobulin (IVIG) treatment for Kawasaki disease (KD). METHODS: This was a single-center analysis of the medical records of 588 patients with KD who had been admitted to Asan Medical Center between 2006 and 2014. Related clinical and laboratory data were analyzed by univariate and multivariate logistic regression analyses. RESULTS: Eighty (13.6%) of the 588 patients with KD were unresponsive to the initial IVIG treatment and received a second dose. For these 80 patients, univariate analysis of the laboratory results obtained before administering the second-line IVIG treatment showed that white blood cell count, neutrophil percent, hemoglobin level, platelet count, serum protein level, albumin level, potassium level, and C-reactive protein level were significant predictors. The addition of methyl prednisolone to the second-line regimen was not associated with treatment response (odds ratio [OR], 0.871; 95% confidence interval [CI], 0.216–3.512; P=0.846). Multivariate analysis revealed serum protein level to be the only predictor of unresponsiveness to the second-line treatment (OR, 0.160; 95% CI, 0.028–0.911; P=0.039). Receiver operating characteristic curve analysis to determine predictors of unresponsiveness to the second dose of IVIG showed a sensitivity of 100% and specificity of 72% at a serum protein cutoff level of <7.15 g/dL. CONCLUSION: The serum protein level of the patient prior to the second dose of IVIG is a significant predictor of unresponsiveness. The addition of methyl prednisolone to the second-line regimen produces no treatment benefit.
Blood Proteins ; C-Reactive Protein ; Chungcheongnam-do ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Leukocyte Count ; Logistic Models ; Medical Records ; Mucocutaneous Lymph Node Syndrome* ; Multivariate Analysis ; Neutrophils ; Platelet Count ; Potassium ; Prednisolone ; ROC Curve ; Sensitivity and Specificity

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.
Adult ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Extracorporeal Membrane Oxygenation ; Follow-Up Studies ; Heart ; Heart Transplantation ; Hemodynamics ; Humans ; Infant* ; Oxygenators, Membrane* ; Tissue Donors

BACKGROUND/AIMS: The purpose of our study was to investigate the change in incidence of intestinal metaplasia (IM) in healthy, young adults over 10 years. MATERIALS AND METHODS: Urease test and histopathology by endoscopic biopsies were performed from volunteers between 1995 and 2005. Histopathological grade was assessed using the updated Sydney System. RESULTS: In total, 714 subjects with a median age of 22.4 years were enrolled. Helicobacter pylori was observed at the antrum and body in 44.5% and 35.1%, respectively. IM limited to the antrum was present in 1.1% of the subjects. The degree of IM correlated negatively with age (P=0.04) but there was no correlation with H. pylori levels or the degree of chronic or active gastritis. Compared to the beginning of the study period, the positivity rate at the end of the study period droped to 45%. IM incidence did not change over the 11-year study period, whereas H. pylori-positivity and the frequency of chronic and active gastritis in the antrum and body dropped significantly over this period (P<0.05). CONCLUSIONS: This result suggests that other factors, besides chronic H. pylori infection or degree of gastritis, may contribute to the progression of atrophy to IM in healthy, young adults.
Adolescent* ; Atrophy ; Biopsy ; Gastritis ; Helicobacter pylori* ; Helicobacter* ; Humans ; Incidence ; Metaplasia* ; Urease ; Volunteers ; Young Adult