Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias. Although most neonatal arrhythmias are asymptomatic and rarely life-threatening, the prognosis depends on the early recognition and proper management of the condition in some serious cases. Precise diagnosis with risk stratification of patients with nonbenign neonatal arrhythmia is needed to reduce morbidity and mortality. In this article, I review the current understanding of the common clinical presentation, etiology, natural history, and management of neonatal arrhythmias in the absence of an underlying congenital heart disease.
Arrhythmia, Sinus ; Arrhythmias, Cardiac* ; Atrial Premature Complexes ; Diagnosis* ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Mortality ; Natural History ; Prognosis ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Ventricular Premature Complexes

A 16-year-old patient with pectus excavatum visited our hospital because of palpitation. He underwent first Nuss operations at the age of 3. When he was 13 years old, the slow-fast type atrioventricular nodal reentrant tachycardia was documented during electrophysiology study. However, the catheter ablation was not conducted because of recurrent atrial fibrillation during procedure. At that time, second Nuss operation was performed due to progressive chest wall deformity. And then, atrioventricular nodal reentrant tachycardia was successfully treated by radiofrequency catheter ablation at the higher position than usual slow pathway zone under the modified fluoroscopic view with the cranial angle although distorted right atrial geometry and radiographic obstacle of Nuss operation bar. The concern about abnormal cardiac and electrical anatomy, and the accurate and modified procedure technique are essential in patients with pectus excavatum. (Ewha Med J 2022;45(3):e6)

Cardiac rhabdomyomas are typically presented in the tuberous sclerosis. Although benign and often associated with spontaneous regression, in rare circumstances huge mass size and critical location can lead to heart failure, ventricular outflow tract obstruction and refractory tachyarrhythmias. An 1-day-old girl was diagnosed as cardiac tumor during perinatal period. At birth, transthoracic echocardiography revealed huge cardiac mass located in septal area of both ventricle measuring 34×30 mm. It protruded into the left ventricular (LV) outflow tract, potentially obstructing it. A surface ECG revealed atrial tachycardia with nonsustained ventricular tachycardia with heart rate of 217 beats per min. The tachyarrhythmias were controlled with intravenous amiodarone. Reduction of the giant cardiac mass was treated with mammalian target of rapamycin pathway inhibitor sirolimus. However, she unfortunately died at 10 days-old because of sudden cardiac arrest maybe due to LV outflow tract obstruction during therapy. Gene analysis revealed TSC2 mutation after death. (Ewha Med J 2022;45(3):e5)

PURPOSE: We evaluated the easily-assessable risk and prognostic factors of septic shock in children with neutropenic fever (NF) which developed after anticancer chemotherapy. METHODS: We retrospectively reviewed the medical records and laboratory data of 97 children who received anticancer chemotherapy at Dong-A University Hospital and had NF between March, 1993 and February, 2001. RESULTS: There were 223 episodes of NF in 97 children, of which 71 episodes (31.8%) of bacteremia and 18 episodes (8.1%) of septic shock developed. The incidence of septic shock was associated with fever duration (> 5 days, odds ratio=7.367, P=0.0159), tachycardia (odds ratio=11.857, P=0.0001), and serum bicarbonate level (<24 mEq/L, odds ratio=6.235, P=0.0378), whereas not with the underlying disease, age, sex, causative organisms, absolute neutrophil count, absolute monocyte count, absolute phagocyte count, the presence of central venous catheter, the accompanied focal infection, and the use of hematopoietic growth factor. Septic shock developed 3.2 (1~11) days after NF. The prognosis of septic shock patients was not associated with the therapeutic timing, antibiotics, fever duration, tachycardia, or serum bicarbonate level. CONCLUSION: We suggest that the fever duration, tachycardia, and serum bicarbonate level could be important risk factors of septic shock in children with NF. Although We could not reveal the prognostic factors in children with septic shock, further studies will be required.
Anti-Bacterial Agents ; Bacteremia ; Central Venous Catheters ; Child ; Drug Therapy ; Fever* ; Focal Infection ; Humans ; Incidence ; Medical Records ; Monocytes ; Neutropenia ; Neutrophils ; Phagocytes ; Prognosis ; Retrospective Studies ; Risk Factors* ; Shock, Septic* ; Tachycardia

PURPOSE: Human adenovirus infection mimics Kawasaki disease (KD) but can be detected in KD patients. The aim of this study was to determine the clinical differences between KD with adenovirus infection and only adenoviral infection and to identify biomarkers for prediction of adenovirus-positive KD from isolated adenoviral infection. METHODS: A total of 147 patients with isolated adenovirus were identified by quantitative polymerase chain reaction. In addition, 11 patients having KD with adenovirus, who were treated with intravenous immunoglobulin therapy during the acute phase of KD were also evaluated. RESULTS: Compared with the adenoviral infection group, the KD with adenovirus group was significantly associated with frequent lip and tongue changes, skin rash and changes in the extremities. In the laboratory parameters, higher C-reactive protein (CRP) level and presence of hypoalbuminemia and sterile pyuria were significantly associated with the KD group. In the multivariate analysis, lip and tongue changes (odds ratio [OR], 1.416; 95% confidence interval [CI], 1.151–1.741; P=0.001), high CRP level (OR, 1.039; 95% CI 1.743–1.454; P= 0.021) and sterile pyuria (OR 1.052; 95% CI 0.861–1.286; P=0.041) were the significant predictive factors of KD. In addition, the cutoff CRP level related to KD with adenoviral detection was 56 mg/L, with a sensitivity of 81.8% and a specificity of 75.9%. CONCLUSION: Lip and tongue changes, higher serum CRP level and sterile pyuria were significantly correlated with adenovirus-positive KD.
Adenoviridae ; Adenoviridae Infections ; Adenovirus Infections, Human ; Biomarkers ; C-Reactive Protein ; Discrimination (Psychology) ; Exanthema ; Extremities ; Humans ; Hypoalbuminemia ; Immunization, Passive ; Lip ; Mucocutaneous Lymph Node Syndrome ; Multivariate Analysis ; Polymerase Chain Reaction ; Pyuria ; Sensitivity and Specificity ; Tongue

An irregular heart rhythm is a common concern among children. Recognizing the cause of the irregular rhythm is crucial for the proper diagnosis and management by primary physicians as well as pediatric specialists. The cause of pediatric arrhythmias can be identified based on the clinical history, physical examination, presenting symptoms, and electrocardiogram. Pediatric arrhythmias are classified as benign and non-benign. Both benign and non-benign arrhythmias can originate from the sinoatrial node, atrial myocardium, atrioventricular node, and ventricle myocardium. The common form of benign arrhythmias, including sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and Wenckebach atrioventricular block, have no clinical significance or need for therapy. On the other hand, non-benign arrhythmias, such as supraventricular tachycardia, ventricular tachycardia, high-degree atrioventricular conduction abnormalities, and genetic arrhythmia, can affect the hemodynamic state and may cause a serious condition in pediatric patients. Most cases of benign arrhythmia are asymptomatic, and the prognosis is favorable for the normal heart. In this article, we review our current understanding of the electrocardiographic characteristics, clinical presentation, etiology, and natural history of benign arrhythmias in pediatric patients.

Left isomerism is characterized by bilateral left-sidedness and multiple associated cardiac and visceral anomalies. The clinical manifestation of left isomerism mainly depends upon the cardiac lesions. Occasionally an individual will have a normal heart and be presented with the extracardiac anomalies. A 3-year-old girl with a diagnosis of left isomerism was presented with pulmonary hypertension and intermittent hypoglycemia. Computerized tomography of the abdomen revealed absence of the portal vein and portosystemic shunt. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained the left renal vein and hemiazygos vein. Her pulmonary hypertension was considered as a consequence of the portosystemic shunt. We report a case of left isomerism in association with absence of the portal vein and a review of literatures.
Abdomen ; Child, Preschool ; Diagnosis ; Female ; Heart ; Humans ; Hypertension, Pulmonary* ; Hypoglycemia ; Isomerism* ; Liver ; Portal Vein* ; Portasystemic Shunt, Surgical ; Renal Veins ; Splenic Vein ; Veins

OBJECTIVE: This study was conducted to investigate the reliability and validity of the Korean version of Autism-Spectrum Quotient (AQ). METHODS: 20 participants with high-functioning autism (HFA) and 99 normal participants were recruited. All participants were completed the AQ and Empathy Quotient (EQ), and parents of the HFA group completed the parent-report AQ. For testing the reliability, we examined Cronbach’s alpha, performed item analysis, and compared self versus parent report score of HFA participants. For testing the validity, we compared the difference of the score of AQ among HFA and control group using independent t-tests, and performed correlation analysis between AQ and EQ. The receiver operation characteristic curve analysis was performed to determine a cut-off. RESULTS: The Korean version of the AQ exhibited adequate internal consistency, and in most items, the HFA group scored higher in comparison to the control group. It was demonstrated that AQ has good discriminant validity through the confirmation of the significant difference in the AQ score between two groups. The concurrent validity was established through the significant correlation between AQ and EQ in the HFA group. The best estimate cut-off score of AQ for screening was 23. CONCLUSION: The Korean version of the AQ was determined as a reliable and valid instrument to assess HFA in Korean population.
Autism Spectrum Disorder ; Autistic Disorder ; Empathy ; Humans ; Mass Screening ; Parents ; Reproducibility of Results*