Newly Developed Sex-Specific Z Score Model for Coronary Artery Diameter in a Pediatric Population

Background@#This study aimed to generate a Z score calculation model for coronary artery diameter of normal children and adolescents to be adopted as the standard calculation method with consensus in clinical practice. @*Methods@#This study was a retrospective, multicenter study that collected data from multiple institutions across South Korea. Data were analyzed to determine the model that best fit the relationship between the diameter of coronary arteries and independent demographic parameters. Linear, power, logarithmic, exponential, and square root polynomial models were tested for best fit. @*Results@#Data of 2,030 subjects were collected from 16 institutions. Separate calculation models for each sex were developed because the impact of demographic variables on the diameter of coronary arteries differs according to sex. The final model was the polynomial formula with an exponential relationship between the diameter of coronary arteries and body surface area using the DuBois formula. @*Conclusion@#A new coronary artery diameter Z score model was developed and is anticipated to be applicable in clinical practice. The new model will help establish a consensus-based Z score model.

Change in Severity and Clinical Manifestation of MIS-C Over SARSCoV-2 Variant Outbreaks in Korea

Background@#There is difference in the incidence of multi-system inflammatory syndrome in children (MIS-C) in patients with different variants of severe acute respiratory syndrome coronavirus 2, however, little is known about the epidemiology in Asian countries. We investigated and compared the epidemiology of the MIS-C during omicron-dominant period with that of previous periods in South Korea. @*Methods@#We obtained clinical, epidemiological and laboratory data on MIS-C cases from national MIS-C surveillance in South Korea. We defined pre-delta period as January 2020–May 2021; delta period as June 2021–December 2021; and omicron period as January 2022–April 2022. We describe the clinical characteristics and outcomes of MIS-C patients by period. @*Results@#A total of 91 cases were assessed to be MIS-C cases. Number of MIS-C cases have increased from six cases during pre-delta period to 66 cases during omicron period, while the incidence rate (the number of MIS-C cases per 100,000 cases of reported coronavirus disease 2019) has decreased from 38.5 cases per 100,000 (95% confidence interval [CI], 14.1–83.9) during pre-delta period to 1.6 cases per 100,000 (95% CI, 1.2–2.0) during omicron periods. During pre-delta period, 66.7% and 100% had hypotension and gastrointestinal involvement, respectively; while during omicron period, 12.1% and 6.1% had such clinical manifestations. Fifty percent of pre-delta MIS-C patients were taken intensive care unit (ICU) cares, while 10.6% of patients during omicron periods were in ICUs. @*Conclusion@#Omicron period were associated with less severe clinical manifestation compared to pre-delta and delta periods. Although incidence rate of MIS-C was lower for the omicron period than pre-delta and delta periods, number of patients reported with MIS-C may pose a substantial clinical burden.

A child with Kawasaki disease and genetic warfarin sensitivity from CYP2C9 and VKORC1 gene variants

Kawasaki disease (KD) is a common febrile disease in East Asia. Because KD with coronary artery aneurysm (CAA) may predispose to thrombosis, children with KD-associated CAA may need anticoagulation in addition to aspirin. In this report, we describe a 6-year-old girl with KD and CAA who was found to have unexpected warfarin-induced coagulopathy caused by CYP2C9 and VKORC1 genotype variants, which affect warfarin metabolism.

A child with Kawasaki disease and genetic warfarin sensitivity from CYP2C9 and VKORC1 gene variants

Kawasaki disease (KD) is a common febrile disease in East Asia. Because KD with coronary artery aneurysm (CAA) may predispose to thrombosis, children with KD-associated CAA may need anticoagulation in addition to aspirin. In this report, we describe a 6-year-old girl with KD and CAA who was found to have unexpected warfarin-induced coagulopathy caused by CYP2C9 and VKORC1 genotype variants, which affect warfarin metabolism.

Corrigendum: Cardiac function associated with home ventilator care in Duchenne muscular dystrophy / 소아과

In this paper, the fourth author’s name and institution were given incorrectly.

Iron deficiency anemia as a predictor of coronary artery abnormalities in Kawasaki disease / 소아과

PURPOSE: Coronary artery abnormalities (CAA) are the most important complications of Kawasaki disease (KD). Iron deficiency anemia (IDA) is a prevalent micronutrient deficiency and its association with KD remains unknown. We hypothesized that presence of IDA could be a predictor of CAA. METHODS: This retrospective study included 173 KD patients, divided into 2 groups according to absence (group 1) and presence (group 2) of CAA. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using a logistic regression model to estimate the association between CAA and other indicators. Due to collinearity between indicators of IDA, each indicator was paired with anemia in 3 models. RESULTS: Serum iron, iron saturation, and ferritin concentration, the 3 indicators of IDA, were significantly higher in group 1 than in group 2. Three sets of models including anemia with iron indicators produced the OR of CAA of 3.513, 3.171, and 2.256, respectively. The 3 indicators of IDA were negatively associated with CAA, by OR of 0.965, 0.914, and 0.944, respectively. The areas under the curve (AUCs) of ferritin concentration, iron saturation, serum iron, anemia, and Kobayashi score were 0.907 (95% CI, 0.851–0.963), 0.729 (95% CI, 0.648–0.810), 0.711 (95% CI, 0.629–0.793), 0.638 (95% CI, 0.545–0.731), and 0.563 (95% CI, 0.489–0.636), respectively. CONCLUSION: Indicators of IDA, especially ferritin, were highly associated with CAA; therefore, they were stronger predictors of CAA than Kobayashi scores. IDA indicators can be used to predict CAA development and to suggest requirements for early interventions.

Infliximab, Is It Really a New Horizon for the Treatment of Kawasaki Disease?

No abstract available.

Do We Know the Normal Blood Pressure Level in Korean Children and Adolescents?

No abstract available.

Cardiac function associated with home ventilator care in Duchenne muscular dystrophy / 소아과

PURPOSE: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. METHODS: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. RESULTS: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 years) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038±620.41 mL) than the nonventilator group (1,455±603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. CONCLUSION: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.

Myocardial Layer-Specific Strain Analysis in Children with Mitochondrial Disease

PURPOSE: Children with mitochondrial disease (MD) have clinical phenotypes that are more severe than those found in adults. In this study, we assessed cardiac function in children with MD using conventional and advanced echocardiographic measurements, explored any unique patterns present, and investigated the development of early cardiomyopathy (CMP). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 33 children with MD. All patients underwent transthoracic echocardiography with conventional and advanced myocardial analysis. We compared all data between patients and an age-matched healthy control group. RESULTS: Conventional echocardiographic diastolic measurements of mitral E, E/A, and tissue Doppler E′ were significantly lower and E/E′ was significantly higher in children with MD, compared with the measurements from the control group. There was no significant difference in longitudinal and radial strain between the groups. Circumferential strain in the endocardium (p=0.161), middle myocardium (p=0.008), and epicardium (p=0.042) were lower in patients, compared to the values in controls. Circumferential strain was correlated with E′ (p 0.60). CONCLUSION: In children with MD, myocardial circumferential strain may develop early in all three layers, even with normally preserved longitudinal and radial strain. This may be an early diagnostic indicator with which to predict CMP in this patient population.

Relationship between serum sodium level and coronary artery abnormality in Kawasaki disease / 소아과

PURPOSE: Kawasaki disease (KD) is an immune-related multisystemic vasculitis that occurs in children, especially ensuing from a coronary artery abnormality. Sodium level is known to be related to vascular injury, which could affect the progress of KD. The purpose of this study was to determine the serum sodium levels that could predict the occurrence of cardiac and coronary artery events in KD. METHODS: We conducted a retrospective review of medical records for 104 patients with KD from January 2015 to December 2015. Patients with serum Na levels of <135 mEq/L at the time of initial diagnosis were assigned to the hyponatremia group. Laboratory findings and echocardiographic data were analyzed for various aspects. RESULTS: Among the 104 patients with KD, 91 were included in the study, of whom 48 (52.7%) had hyponatremia. The degree of fever, white blood cell count, percentage of neutrophils, percentage of lymphocytes, total bilirubin level, brain natriuretic peptide level, erythrocyte sedimentation rate, and C-reactive protein level were higher in the patients with hyponatremia. They also demonstrated a trend of larger coronary artery diameters based on Z scores. CONCLUSION: The severity of vascular inflammation in acute KD with hyponatremia might worsen the prognosis of coronary vasculature. Although no statistically significant correlation was found between the initial serum sodium levels and coronary arteriopathy in the patients with KD in this study, a long-term follow-up study with a larger number of enrolled patients should be designed in the future to elucidate the relationship between serum sodium level and coronary arteriopathy in patients with KD.

Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

PURPOSE: As cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction. MATERIALS AND METHODS: We reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5-18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5-17 years). RESULTS: Epicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p<0.001), myocardium (DMD: -12.7±3.8%; control: -18.0±4.0%; p<0.001), and epicardium (DMD: -8.4±4.0%; control: -12.2±5.0%; p=0.006) were significantly decreased in DMD. Circumferential SRs were lower in the endocardial (DMD: -1.46±0.38; control: -1.78±0.27; p=0.002) and myocardial layers (DMD: 1.02±0.27; control: -1.28±0.22; p=0.001). CONCLUSION: In DMD patients, deteriorations in myocardial circumferential strain might be an indicator for predicting cardiomyopathy.

Myocardial Tissue Doppler Velocity in Child Growth

BACKGROUND: In adults, tissue Doppler imaging (TDI) is a recommended component of routine echocardiography. However, TDI velocities are less accepted in pediatrics, due to their strong variability and age dependence in children. This study examines the distribution of myocardial tissue Doppler velocities in healthy children to assess the effect of age with cardiac growth on the various echocardiographic measurements. METHODS: Total 144 healthy children were enrolled in this study. They were recruited from the pediatric outpatient clinic for routine well-child visits. The statistical relationships between age and TDI values were analyzed. Also, the statistical relationships between body surface area (BSA) and TDI values, left ventricle end-diastolic dimension (LVEDD) and TDI values were analyzed. Also, we conducted multivariate analysis of cardiac growth parameters such as, age, BSA, LVEDD and TDI velocity data. RESULTS: All of the age, BSA, and LVEDD had positive correlations with deceleration time (DT), pressure half-time (PHT), peak early diastolic myocardial velocity, peak systolic myocardial velocity, and had negative correlations with peak late diastolic velocity (A) and the ratio of trans-mitral inflow velocity to early diastolic velocity of mitral annulus (E/E'). In the multivariate analysis, all of the age, BSA, and LVEDD had positive correlations with DT, PHT, and negative correlations with A and E/E'. CONCLUSION: The cardiac growth parameters related alterations of E/E' may suggest that diastolic myocardial velocities are cardiac growth dependent, and diastolic function has positive correlation with cardiac growth in pediatric group. This cardiac growth related myocardial functional variation would be important for assessment of cardiac involvement either in healthy and sick child.

Myocardial Rotation and Torsion in Child Growth

BACKGROUND: The speckle tracking echocardiography can benefit to assess the regional myocardial deformations. Although, previous reports suggested no significant change in left ventricular (LV) torsion with aging, there are certain differences in LV rotation at the base and apex. The purpose of this study was to evaluate the change and relationship of LV rotation for torsion with aging in children. METHODS: Forty healthy children were recruited and divided into two groups of twenty based on whether the children were preschool-age (2–6 years of age) or school-age (7–12 years of age). After obtaining conventional echocardiographic data, apical and basal short axis rotation were assessed with speckle tracking echocardiography. LV rotation in the basal and apical short axis planes was determined using six myocardial segments along the central axis. RESULTS: Apical and basal LV rotation did not show the statistical difference with increased age between preschool- and school-age children. Apical radial strain showed significant higher values in preschool-age children, especially at the anterior (52.8 ± 17.4% vs. 34.7 ± 23.2%, p < 0.02), lateral (55.8 ± 20.4% vs. 36.1 ± 22.7%, p < 0.02), and posterior segments (57.1 ± 17.6% vs. 38.5 ± 21.7%, p < 0.01). The torsion values did not demonstrate the statistical difference between two groups. CONCLUSION: This study revealed the tendency of higher rotation values in preschool-age children than in school-age children. The lesser values of rotation and torsion with increased age during childhood warrant further investigation.

Transcatheter Treatment of Atrial Septal Defect Presenting with Platypnea-Orthodeoxia Syndrome

A 29-year-old woman was referred to our institute for symptomatic hypoxemia. Her dyspnea was aggravated while sitting or standing and relieved while in supine position. She did not have any pulmonary disease. Transthoracic echocardiography and heart computed tomography revealed an underestimated small atrial septal defect (ASD) with a left-to-right shunt. A cardiac catheterization was performed to evaluate pulmonary hypertension. It revealed a normal pulmonary artery pressure and a large ASD with bidirectional shunt during Valsalva maneuver by intracardiac echocardiogram. Her arterial oxygen saturation decreased from 93% while supine to 79% while at a 15degrees sitting position. Thus, the patient was diagnosed with platypnea-orthodeoxia syndrome. The ASD was successfully closed with Amplatzer(R) (St. Jude Medical) septal occluder and both platypnea and orthodeoxia were resolved immediately after the procedure.

Do We Understand Cardiovascular Issues in Children and Adolescents with Metabolic Syndrome?

No abstract available.

Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy / 소아과

PURPOSE: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. METHODS: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. RESULTS: The LV mass-for-height z scores were significantly below normal in children with MD (-1.02+/-1.52, P<0.001) or DMD (-0.82+/-1.61, P=0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R=0.377, P<0.001) and those with DMD (R=0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R=0.462, P<0.001) and cardiac index (R=0.358, P<0.001). CONCLUSION: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients.

Retraction: Aortic Dissection and Rupture in a Child

No abstract available.

The Study of Characteristics of Cutaneous Manifestations of Kawasaki Disease in Korean Patients (2006-2010) / 대한피부과학회지

BACKGROUND: Kawasaki disease (KD) is an acute inflammation of middle-sized arteries such as the coronary artery. KD is an important disease due to its high mortality rate, which approaches 1-2% without appropriate treatment. Since the various mucocutaneous lesions of KD are related to the dermatologic field, dermatologists should take an interest in this disorder and understand the lesions. OBJECTIVE: To evaluate the characteristics of cutaneous lesions in Korean patients with KD. METHODS: A total of 919 patients with KD were assessed at the department of pediatric cardiology and dermatology, Myongji hospital between January 2006 and December 2010. RESULTS: Of the 919 patients, 566 (61.6%) were diagnosed with typical KD and 353 (38.4%) with atypical KD. KD was most frequent in the 4 year-old group (22.7%), and the ratio of male to female was 1.5:1. Among the mucocutaneous manifestations, erythema of the oropharyngeal mucosa (75.7%) occurred most frequently, followed by polymorphous trunk rash (41.7%), and changes of palms and soles (25.6%). A total of 606 patients (65.9%) with KD had associated diseases as follows: pneumonia (211 patients; 34.8%), pharyngotonsilitis (168 patients; 27.7%), gastroenteritis (132 patients; 21.8%), asthma (15 patients, 2.5%), atopic dermatitis (11 patients, 1.8%). CONCLUSION: Our study shows that change in the lips or oral cavity is the most common mucocutaneous manifestation in both classic and atypical KD. Also, morbiliform rash is the most common pattern of polymorphous rashes. Interestingly, erythema multiforme-like lesions occupied accounted for a considerable portion of KD cases compared to previous studies.

Aortic Dissection and Rupture in a Child

After developing sudden severe chest pain, an 11-year-old boy presented to the emergency room with chest pain and palpitations and was unable to stand up. The sudden onset of chest pain was first reported while swimming at school about 30 minutes prior to presentation. Arterial blood pressure (BP) was 150/90 mmHg, heart rate was 120/minute, and the chest pain was combined with shortness of breath and diaphoresis. During the evaluation in the emergency room, the chest pain worsened and abdominal pain developed. An aortic dissection was suspected and a chest and abdomen CT was obtained. The diagnosis of aortic dissection type B was established by CT imaging. The patient went to surgery immediately with BP control. He died prior to surgery due to aortic rupture. Here we present this rare case of aortic dissection type B with rupture, reported in an 11-year-old Korean child.