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To analyze the clinical presentation and outcomes of myocarditis after administration of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) messenger RNA (mRNA) vaccine. Nine case series and 15 case reports (74 patients) of myocarditis after administration of the BNT162b2 or mRNA-1273 vaccine were reviewed from PubMed, Scopus, Embase, and Web of Science. We analyzed clinical manifestations, diagnostic findings, and outcomes. In addition, we performed a pooled analysis and investigated risk factors leading to admission to the intensive care unit and recovery with conservative care. Most patients were male (94.6%), and the median age (range) was 17.6 (14–70) years. Patients who received the BNT162b2 (n = 58, 78.4%) vaccine presented fewer systemic symptoms and left ventricular dysfunction than mRNA-1273 recipients. Although patients under 20 years experienced more fever and myalgia, they had better ejection fraction and less prominent myocardial inflammation in magnetic resonance imaging than older patients. The clinical course of all patients was favorable without mortality, and one-third of patients resolved with conservative care alone. Risk factor analyses revealed that patients with gastrointestinal symptoms required intensive care (odds ratio: 20.3, 95% confidence interval 1.90–217, p = 0.013). The risk of fatality in myocarditis subjected to mRNA vaccination seems to be low. However, patients with gastrointestinal symptoms received more intensive care, and a significant proportion of patients recovered with conservative management.

Background and Objective: Incomplete Kawasaki disease (KD) has often been misdiagnosed due to a lack of the clinical manifestations of classic KD. However, it is associated with a markedly higher prevalence of coronary artery lesions. Identifying coronary artery lesions by echocardiography is important for the timely diagnosis of and favorable outcomes in KD. Moreover, similar to KD, coronavirus disease 2019, currently causing a worldwide pandemic, also manifests with fever; therefore, it is crucial at this moment that KD should be distinguished clearly among the febrile diseases in children. In this study, we aimed to validate a deep learning algorithm for classification of KD and other acute febrile diseases. Methods: We obtained coronary artery images by echocardiography of children (n = 88 for KD; n = 65 for pneumonia). We trained six deep learning networks (VGG19, Xception, ResNet50, ResNext50, SE-ResNet50, and SE-ResNext50) using the collected data. Results: SE-ResNext50 showed the best performance in terms of accuracy, specificity, and precision in the classification. SE-ResNext50 offered a precision of 76.35%, a sensitivity of 82.64%, and a specificity of 58.12%. Conclusions: The results of our study suggested that deep learning algorithms have similar performance to an experienced cardiologist in detecting coronary artery lesions to facilitate the diagnosis of KD.

A concerning development during the coronavirus disease pandemic has been multisystem inflammatory syndrome in children. Reports of this condition in East Asia have been limited. In South Korea, 3 cases were reported to the national surveillance system for multisystem inflammatory syndrome in children. All case-patients were hospitalized and survived with no major disease sequelae.

BACKGROUND Incomplete Kawasaki disease (KD) has often been misdiagnosed due to a lack of the clinical manifestations of classic KD. However, it is associated with a markedly higher prevalence of coronary artery lesions. Identifying coronary artery lesions by echocardiography is important for the timely diagnosis of and favorable outcomes in KD. Moreover, similar to KD, coronavirus disease 2019, currently causing a worldwide pandemic, also manifests with fever; therefore, it is crucial at this moment that KD should be distinguished clearly among the febrile diseases in children. OBJECTIVE In this study, we aimed to validate a deep learning algorithm for classification of KD and other acute febrile diseases. METHODS We obtained coronary artery images by echocardiography of children (n = 88 for KD; n = 65 for pneumonia). We trained six deep learning networks (VGG19, Xception, ResNet50, ResNext50, SE-ResNet50, and SE-ResNext50) using the collected data. RESULTS SE-ResNext50 showed the best performance in terms of accuracy, specificity, and precision in the classification. SE-ResNext50 offered a sensitivity of 82.64% and a specificity of 58.12%. CONCLUSIONS The results of our study suggested that deep learning algorithms have similar performance to an experienced cardiologist in detecting coronary artery lesions to facilitate the diagnosis of KD. CLINICALTRIAL none

Transcatheter device closure of a secundum atrial septal defect (ASD) is followed by a noticeable change in the left atrium (LA) and left ventricle (LV) over a long-term follow-up. This study aimed to assess the remodeling of the atrial and ventricular myocardium with respect to diastolic function in adult ASD patients. Around 38 asymptomatic patients (age: 48.6 &plusmn, 17.1 years, range: 23&ndash, 69 years) diagnosed with ASD during routine health check-up and who underwent ASD device closure with the Amplatzer septal occluder were included in this retrospective study, and their medical records (containing echocardiographic data) were reviewed. The defect size was 21.77 + 6.79 mm, while the balloon measurement of the defect was 24.29 &plusmn, 6.64 mm. The patients were divided into two groups, namely, Group I (with 18 patients aged &lt, 50 years [mean: 33.06 &plusmn, 9.43 years] and Group II (with 20 patients aged &ge, 50 years [mean: 62.55 &plusmn, 7.54 years]). Comparison of echocardiographic data collected before closure and at a follow-up 2 years later revealed significant differences between pre-closure and post-closure states in the left ventricular end-diastolic dimension (40.76 &plusmn, 3.28 vs. 43.39 &plusmn, 3.52), left ventricular mass (99.64 &plusmn, 28.81 vs. 116.57 &plusmn, 32.03), and right ventricular pressure (36.88 &plusmn, 12.20 vs. 31.81 &plusmn, 11.11). Tissue Doppler measurements were significantly decreased 2 years after closure, while the post-closure E/E&rsquo, was higher than the pre-closure E/E&rsquo, (11.58 &plusmn, 4.80 vs. 8.79 + 3.19, p &lt, 0.005). In both groups, mitral A and tissue Doppler E&rsquo, A&rsquo, and S&rsquo, values decreased post-closure, while the E/E&rsquo, was higher in Group II than in Group I at both pre-closure and long-term follow-up measurements (pre-closure: 9.60 &plusmn, 5.15 vs. 7.41 &plusmn, 1.42, p &lt, 0.003, post-closure: 13.03 + 4.05 vs. 10.49 &plusmn, 3.95, p &lt, 0.02). The LA size and LV function exhibited recognizable remodeling after transcatheter ASD closure. Because the LV preload elevation (i.e., E/E&rsquo, ) after ASD closure can be pronounced in older patients, caution should be provided more in older patients than in younger patients. Hence, it may be beneficial to close the ASD at an earlier age in adults even after a late diagnosis, a relatively younger age may be more suited for adaptation to the remodeling process to protect the myocardial function. Careful consideration should be given to the previous underloaded left heart after long-term closure.

Background This study aimed to investigate recent epidemiologic features of Kawasaki disease (KD) in South Korea. Methods The ninth triennial nationwide questionnaire survey collected data on the demographic findings, symptoms and signs, treatment patterns and coronary artery complications of acute-phase KD occurred in 2015-2017 from 98 hospitals with pediatric residency programs and 108 community hospitals without residency programs. Results We received data from 93 of the 98 hospitals (response rate: 94.9%) with residency programs and 75 of the 108 community-based children's hospitals (response rate: 69.4%) without residency programs. In the 3-year survey period, a total of 15,378 (5449 in 2015, 5171 in 2016 and 4758 in 2017) cases of KD were reported. The mean age at diagnosis was 33.0 ± 24.8 months (range: 0-205 months), and the male-to-female ratio was 1.41:1. The overall KD incidence was 196.9 (202.2 in 2015, 197.1 in 2016 and 191.0 in 2017) per 100,000 younger than 5 years population. Recurrent cases were 4.85%. KD occurred more frequently during winter (December-January) and late spring (May-June). Intravenous immunoglobulin (IVIG) was administered to 95% of the patients; nonresponder rate for the first IVIG was 14.8%. Coronary artery aneurysms and giant coronary artery aneurysms (internal diameter >8 mm) occurred in 1.7% and 19 patients, respectively. Two patients died due to multiorgan failure and hepatic encephalopathy. Conclusion Peak incidence of KD in South Korea was 202.2 per 100,000 younger than 5 years population (2015), and the incidence of giant coronary artery aneurysm decreased to 0.09% (2017).

Percutaneous pulmonary valve implantation (PPVI) is used to treat pulmonary stenosis (PS) or pulmonary regurgitation (PR). We described our experience with PPVI, specifically valve-in-valve transcatheter pulmonary valve replacement using the Melody valve and novel self-expandable systems using the Pulsta valve. We reviewed data from 42 patients undergoing PPVI. Twenty-nine patients had Melody valves in mostly bioprosthetic valves, valved conduits, and homografts in the pulmonary position. Following Melody valve implantation, the peak right ventricle-to-pulmonary artery gradient decreased from 51.3 &plusmn, 11.5 to 16.7 &plusmn, 3.3 mmHg and right ventricular systolic pressure fell from 70.0 &plusmn, 16.8 to 41.3 &plusmn, 17.8 mmHg. Thirteen patients with native right ventricular outflow tract (RVOT) lesions and homograft underwent PPVI with the new self-expandable Pulsta valve&mdash, a nitinol wire stent mounted with a trileaflet porcine pericardial valve. Following Pulsta valve implantation, cardiac magnetic resonance imaging showed a decreased PR fraction and that the right ventricular end-diastolic volume index decreased from 166.1 &plusmn, 11.9 to 123.6 &plusmn, 12.4 mL/m2. There were no mortality, severe procedural morbidity, or valve-related complications. At the mean 14.2 month (4&ndash, 57 months) follow-up, no patients had more than mild PR. PPVI using Melody and Pulsta valves was first shown to provide excellent early outcomes without serious adverse event in most patients with RVOT dysfunction in Korea.

The purpose of this study was to evaluate and assess the compatibility of current risk scoring systems from Japan that were developed to predict intravenous immunoglobulin (IVIG) resistance in patients with Kawasaki disease (KD). The authors previously investigated another prediction model for patients with refractory KD in Korea. A retrospective study involving 350 patients with KD who were admitted between January 2014 and December 2015 was performed. Patients younger than 2 years were excluded for the propensity score matching in this study. Patients were classified into IVIG responders and IVIG resistance groups. The well-known Harada, Kobayashi, and Egami risk scores were calculated for each patient, and the proportion of high-risk patients was compared between the two groups for each risk score. Logistic regression analysis revealed that platelets, C-reactive protein (CRP) levels, and aspartate aminotransferase levels were independent predictors of IVIG resistance. Multivariate analysis suggested that platelets and CRP were risk factors. Risk-scoring systems from Japan have good specificity but low sensitivity. Among the three risk scoring systems, the Kobayashi risk score demonstrated significant differences between the IVIG resistance and IVIG responder groups in Korean patients with KD. It is very important to identify IVIG-resistant patients to protect them from ongoing coronary arterial lesion(s); therefore, early prediction and timely optimal additional treatment is of significant benefit. It would be helpful to construct a highly sensitive, exclusive scoring system for Korean patients with KD.

To reveal the recent epidemiologic features of Kawasaki disease (KD) in South Korea based on data from a nationwide survey.We collected data between 2012 and 2014 regarding the incidence, symptoms and signs, treatment trends and coronary complications associated with acute KD by sending questionnaires to 97 hospitals with pediatric residency programs as well as 19 community hospitals without residency training.We received full and partial data from 97 and 13 hospitals, respectively (response rate: 94.8%). A total of 14,916 cases of KD were reported by these 110 hospitals (4588 in 2012, 5183 in 2013 and 5145 in 2014). The male-to-female ratio was 1.4:1, and the median age at diagnosis was 29 months. The incidence of KD per 100,000 children younger than 5 years of age were 170.9, 194.9 and 194.7 in 2012, 2013 and 2014, respectively. The recurrence rate was 4.7%. KD occurred more frequently during summer (especially June and July) and winter (December and January) seasons. Intravenous immunoglobulin was administered to 95.4% of the patients, and the nonresponder rate for the first intravenous immunoglobulin was 11.8%. Coronary aneurysm occurred in 1.7% of the patients, and giant aneurysm developed in 19 patients (0.16%) during the 3 years. One patient had myocardial infarction and 1 patient died of suspected coronary aneurysm rupture.The incidence of KD in South Korea increased to 194.7 per 100,000 children younger than 5 years in 2014; meanwhile, the coronary aneurysm rate decreased to 1.7%.

Purpose Coronary artery abnormalities (CAA) are the most important complication of Kawasaki disease (KD). Iron deficiency anemia (IDA) is prevalent micronutrient deficiencies and its association with KD remains unknown. We hypothesized the presence of IDA could be a predictor of CAA. Methods This retrospective study included 173 KD patients, divided into two groups by absence (Group 1) and presence (Group 2) of CAA. The odds ratio (OR) with 95% confidence interval (CI) was calculated using a logistic regression model to estimate the association between CAA and other indicators. Due to the collinearity between the IDA indicators, each indicator was paired with anemia in 3 models. Results The 3 indicators of IDA, serum iron, iron saturation and ferritin, were all significantly higher in Group 1 than in Group 2. Three sets of models including anemia with iron indicators produced the odd ratio (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 Area under the curve (AUC) of ferritin, iron saturation, serum iron, anemia, and Kobayashi score was 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. Figure 1 & 3 Conclusion The indicators of IDA, especially ferritin, were highly associated with CAA, so that they were stronger predictors compared to the Kobayashi score. The IDA indicators can be used to predict CAA development and suggest the need for early intervention. Acknowledgement/Funding None

Background: Syncope is a common problem in children and adolescents. However, a large proportion of syncope cases have no underlying cause.Purpose: This study aimed to identify the factors affecting the severity of syncope using tissue Doppler imaging (TDI).Methods: This retrospective study included 61 children and adolescents with syncope who underwent echocardiography. The head-up-tilt test (HUT) was performed when there was a more severe syncopal event. We compared the echocardiographic findings between the execute HUT and nonexecute HUT, negative HUT result and positive HUT result, and normal electrocardiogram (ECG) and abnormal ECG groups. Data were analyzed using an unpaired t test post hoc analysis.Results: In the execute and nonexecute HUT groups, the odds ratios were 0.55 for medial E/E′ (P=0.040) and 0.64 for lateral E/E′ (P=0.049). Comparison of the results of the decreased, normal, and increased groups for lateral E/E′ revealed a significant difference in the execution HUT and nonexecute HUT groups (overall, P=0.004; decreased vs. increased, P= 0.003; normal vs. increased, P=0.050).Conclusion: Medial E/E′ and lateral E/E′ were decreased in patients with severe syncopal events. These findings suggest that the presence of left ventricular diastolic deterioration may cause hypoperfusion even in the absence of organic causes and, consequently, increase syncope severity and frequency. The TDI measured by echocardiography can be used as an index to predict syncope recurrence and/or severity.

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.

Recently, the prevalence of infant Kawasaki disease (KD) has increased. However, the myocardial functional analysis of infant KD can be difficult and rarely reported. The purpose of this study was to investigate layer specific myocardial strain analysis for better assessment of the acute period in infant KD. The study retrospectively reviewed the echocardiographic data of 25 infant patients with KD at the acute phase. With advanced imaging, pulsed tissue Doppler velocity data, myocardial strain with three layers specific analysis was performed. Then the data were compared with 25 age-matched healthy control infants. The measures of longitudinal strain and radial strain were decreased in infant KD compared to healthy controls. The circumferential strain was significantly decreased in infant KD at all three myocardial layers, especially in the endocardial layer (KD: −20.5 ± 6.4 % vs. control: −25.6 ± 7.6 %, endocardium, p = 0.00001; −14.6 ± 4.4 % vs. −18.1 ± 4.0 %; middle myocardium, p = 0.01; −9.7 ± 3.3 % vs. −11.4 ± 3.8 %; epicardium, p = 0.04). The acute phase of infant KD demonstrated decreased myocardial strain measurement. Circumferential strain was the lowest in the endocardial layer. Further continuous long-term follow up for myocardial assessment should be recommended even after recovery with appropriate treatment.

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.

Timely diagnosis of coronary involvement is paramount in Kawasaki disease (KD) as it can be associated with long-term morbidity. However, echocardiographic measurements of coronary artery dilation in KD are inconsistent and not proficient for all abnormal arteries. The purpose of this study was to investigate more valuable indices and determine their sensitivity and specificity for early diagnosis of coronary involvement in KD. We performed this retrospective study in 218 children. All patients underwent laboratory and echocardiographic evaluations upon admission. We measured the size of the left main coronary artery (LMA), left anterior descending coronary artery (LAD), right coronary artery (RCA), and aorta (Ao), and calculated the LMA/Ao, LAD/Ao, and RCA/Ao ratios. We also calculated the cut-off values of each index using receiver operating characteristic curves. LMA, LAD, and RCA measurements did not correlate with white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, or brain natriuretic peptide level. The LMA measurement was associated with hemoglobin, hematocrit, and iron saturation. LAD/Ao was correlated with white blood cell and platelet counts (P

The purpose of this study was to investigate Korean elite soccer players’ myocardial function using the conventional and advanced speckle tracking imaging to compare the difference with the normal controls. We used 2D echocardiography speckle tracking echocardiography (STE) to evaluate LV regional strain in 29 elite soccer players compared to 29 age-matched healthy controls. Conventional, tissue Doppler, and STI echocardiography was performed, for strain at base and apex, rotation and torsion. There is no difference in longitudinal strain (−17.6 ± 1.8 vs −17.3 ± 2.9, p = ns), and basal radial strain. However, the significant increases were noticed in basal circumferential strain (−17.5 ± 2.6 vs −15.5 ± 8.9, p = 0.05), apical radial strain (33.1 ± 20.5 vs 22.5 ± 19.4, p = 0.02), and apical circumferential strain in soccer players (−21.4 ± 4.8 vs −16.8 ± 7.6, p = 0.005). Soccer players showed the higher rotation at base (−3.9 ± 1.9 vs −2.6 ± 3.2, p = 0.03), and apex (6.98 ± 2.62 vs 6.21 ± 3.81, p = 0.05), higher torsion (10.9 ± 3.7 vs 8.8 ± 6.3, p = 0.05). In conclusion, the elite soccer players’ heart demonstrated the unique ventricular adaptation. These alterations could benefit the cardiovascular adjustment to exercise without much loss of myocardial energy expenditure.

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.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.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) (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.

We report a fatal case of acute gastric dilatation in a 3-year old boy who presented with severe abdominal pain and massive gastric distension in the emergency room. On physical examination the patient was in shock due to acute abdomen and lower limb ischemia. Initial laboratory findings showed multi-organ failure with acute renal failure and pancreatitis. Abdominal computed tomography (CT) showed marked dilatation of the stomach resulting in compression of the abdominal aorta. The left kidney, spleen and pancreas were not visible on CT due to the necrotic changes. The patient was quickly stabilized by initial volume resuscitation, but suddenly deteriorated immediately after gastric decompression via nasogastric tube, and died from multi-organ failure 3 h after initial presentation.

Erythromelalgia is a rare clinical syndrome characterized by erythema, increased temperature, and severe burning pain that can be aggravated by warmth or relieved by cold. Erythromelalgia occurs either as a primary, idiopathic form, or secondary to a number of diseases and conditions. Although fairly well studied in adults, the characteristics, pathogenesis, and natural history are poorly characterized in the pediatric age group. Different therapeutic options have been tried, but no optimal treatment has been suggested for erythromelalgia. We report a rare case of linear erythromelalgia in a 12-year-old girl involving her central body from the peripheral extremities, which seemed to be secondary due to vasculitis. Clinical progress waxed and waned on maintenance aspirin and propranolol.

Kawasaki disease (KD), an acute febrile vasculitis, is the most common cause of acquired heart disease in infants and young children. However, the diagnosis of infantile KD can be difficult or delayed due to vague clinical manifestations. This current study aimed to assess the clinical characteristics and cardiac complications of infantile KD. The study retrospectively reviewed the data of 242 patients with KD. The clinical, laboratory, and echocardiographic data between infants and older children were analyzed. The patients were divided into two groups: infants 12 months old or younger and children older than 12 months. The rate of incomplete KD was much more frequent in infants. During all phases, prohormone brain natriuretic peptide (Pro-BNP) levels were higher in infants, as was thrombocytosis. The coronary artery z-score was higher in infants at all phases of KD. On tissue Doppler imaging, the E/E' ratio (ratio of transmitral Doppler early filling velocity to tissue Doppler early diastolic mitral annular velocity) was higher at the septal and lateral annulus in infants. Infant patients with KD are at increased risk for the development of coronary abnormalities and diastolic dysfunction. Higher levels of Pro-BNP and thrombocytosis with diastolic echo parameters of a higher E/E' ratio can help to identify incomplete KD in infants.

Summary We present the case of an 11-year-old boy who developed sudden severe chest pain. When he was referred to the emergency department with this chest pain and palpitation, he could not even stand up. The sudden onset of chest pain was first complained about while he was swimming at school about 30min previously. His arterial blood pressure was 150/90mmHg, heart rate was 120/min, and the chest pain was combined with shortness of breath and diaphoresis. During the evaluation in the emergency room, he complained of much severer chest pain and newly developed abdominal pain. We suspected aortic dissection and took computed tomography (CT) images of his chest and abdomen. The diagnosis of aortic dissection type B was established with the help of CT imaging. The patient went to surgery immediately with blood pressure control. He was lost before the operation because of aortic rupture. We report one rare case of aortic dissection type B with rupture in an eleven-year-old child in Korea.

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′ (p0.60). 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.

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.

REFER TO THE PAGE 10-19 The cardiovascular functional assessment with metabolic syndrome has been reported with many aspects in adult patients. On the contrary, in children and adolescents patients, the metabolic syndrome and other combined issues have not been much investigated. In general, the concept of metabolic syndrome has not been often implicated in pediatric age group, yet. Instead, specific metabolic disease could be diagnosed in case of metabolic pathway problem. However, recently, the importance to inspect metabolic issues including obesity has been emphasized in children and adolescents.1),2),3),4) The potential cardiovascular functional alteration might be the high priority to be considered for these patients, especially for their healthy growth and development.5),6),7),8) For detail evaluation of cardiac function, conventional echocardiographic measurement, tissue Doppler echocardiographic data and strain imaging should be assessed with regional myocardial analysis.9),10) In children and adolescents, their standard values have been established according to their age and body surface area, the measurement data with metabolic syndrome could be compared. In this study, relative ventricular systolic and diastolic functional decrement was noticed in metabolic syndrome group compared to the control group, which is similar to the previous published studies in adults.11) The tissue Doppler echocardiographic E' velocity demonstrated negative correlation with body mass index (BMI), fat %, fat mass, significant positive correlation with high-density lipoprotein-cholesterol. Significant correlation was observed at global longitudinal strain with insulin, homeostasis model of assessment for insulin resistance index and triglyceride, global circumferential strain with weight, waist circumference, fat mass, BMI, and fat %, left ventricular mass with alanine aminotransferase and glupcose. However, the study individuals are just between 14 to 15 years old without specific symptom, their echocardiographic measurements and laboratory data are continued to change with growth. If the yearly follow-up data will be acquired, we would better interpret the characteristics with metabolic pathway issues in this challenging period. Moreover, all the other categories of metabolic syndrome, such as dyslipidemia, diabetes, micro-vascular or macro-vascular disease, mitochondrial disease, should be respectively clarified with the children's growth.1),2),3) Meanwhile, the interesting point of these results is quite parallel to the reports in adult metabolic syndrome patients. Even though, the myocardial function has been preserved within normal range, it would be vulnerable with progress of the underlying metabolic pathway issues. Nevertheless, the growing children and adolescents have much various unique factors for their metabolism rather than grown-up adults, the pattern of cardiovascular characteristics revealed comparable with some metabolic deterioration. According to the results of this study, left ventricular mass index, strain and strain rate are practical values for assessment of ventricular function in children and adolescents metabolic syndrome. These parameters should be recommended to measure for the routine echocardiographic examination in these patients group, so that we might predict their subtle cardiovascular functional alteration over time.

Coronary artery involvement remains the most important complication with Kawasaki disease (KD). Additional myocardial injury can be caused by inflammatory response and ischemic event. However, the long-term outcome of myocardial function has not been fully known in KD. The purpose of this study is to evaluate myocardial function in school-aged children who had the past history of KD. Sixty-seven children in the second grade of elementary schools, who had the past history of KD, were included. Echocardiographic measurements of each coronary artery and myocardial function were obtained as the long-term follow-up data, and compared with the baseline data at the time of initial presentation of KD. The mean age of the subjects was 8.6 ± 2.4 years, and 4.3 ± 3.4 years have passed since the diagnosis of KD. Among the echocardiographic data, interventricular septum thickness at end-diastole (IVSd), LV internal diameters at end-systole (LVIDs), maximal velocity of late diastolic filling across mitral valve (mitral A) flow, maximal velocity of early diastolic filling across mitral valve (mitral E)/A ratio, mitral inflow E wave to peak early diastolic tissue velocity (E/E') ratio showed significant differences between the baseline and follow-up measurements. Coronary Z-score of left main artery (LMA), left anterior descending (LAD), and right coronary artery (RCA) showed no significant difference. The school-aged children with the past history of KD may have diastolic dysfunction. Therefore, appropriate assessment of myocardial function would be recommended during the follow-up period in children with KD.

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

We report a fatal case of acute gastric dilatation in a 3-year old boy who presented with severe abdominal pain and massive gastric distension in the emergency room. On physical examination the patient was in shock due to acute abdomen and lower limb ischemia. Initial laboratory findings showed multi-organ failure with acute renal failure and pancreatitis. Abdominal computed tomography (CT) showed marked dilatation of the stomach resulting in compression of the abdominal aorta. The left kidney, spleen and pancreas were not visible on CT due to the necrotic changes. The patient was quickly stabilized by initial volume resuscitation, but suddenly deteriorated immediately after gastric decompression via nasogastric tube, and died from multi-organ failure 3 h after initial presentation.

Cor triatriatum is a rare congenital anomaly which has wide-ranging severity depending on the structure of the additional intra-atrial septum. We report the case of a boy who was incidentally diagnosed with cor triatriatum by echocardiography during diagnosis and treatment of Kawasaki disease. He has had no definite life-threatening symptoms except recurring respiratory infections. Serial follow-up of such patients is mandatory to check for the potential risks related to the anomalous inter-atrial structure.

Erythromelalgia is a rare clinical syndrome characterized by erythema, increased temperature, and severe burning pain that can be aggravated by warmth or relieved by cold. Erythromelalgia occurs either as a primary, idiopathic form, or secondary to a number of diseases and conditions. Although fairly well studied in adults, the characteristics, pathogenesis, and natural history are poorly characterized in the pediatric age group. Different therapeutic options have been tried, but no optimal treatment has been suggested for erythromelalgia. We report a rare case of linear erythromelalgia in a 12-year-old girl involving her central body from the peripheral extremities, which seemed to be secondary due to vasculitis. Clinical progress waxed and waned on maintenance aspirin and propranolol.

Objective Congestive hepatopathy after a Fontan operation can have a major effect on long-term morbidity. We evaluated congestive hepatopathy in patients with Fontan circulation using transient elastography to determine which risk factors for hepatopathy are related to liver stiffness (LS). Methods We evaluated 46 patients with Fontan circulation and 26 with right side heart failure (RHF) and hepatic congestion using laboratory tests, the aspartate aminotransferase-to-platelet ratio index, ultrasonography, and transient elastography. Results The LS value was significantly greater in the Fontan group (21.1 ± 8.0 kPa) than in the RHF group (10.0 ± 9.0 kPa). The total bilirubin and albumin serum levels, white blood cell count, and aspartate aminotransferase to platelet ratio index correlated significantly with LS in the Fontan group. Of the risk factors, age at evaluation (r = 0.42, P = .004), age at Fontan completion (r = 0.51, P P = .02), and spleen size (r = 0.53, P = .002) correlated significantly with LS. Nineteen patients in the Fontan group (41.3%) had abnormal ultrasound findings, and the frequency of abnormal findings increased with increasing LS ( P = .002). In the subgroup with the greatest LS value (≥30 kPa), 88.9% had abnormal ultrasound findings and 44.4% liver cirrhosis. Multivariate analysis revealed that age at Fontan procedure completion and total bilirubin were independent risk factors for hepatopathy. Conclusions The present study revealed that congestive hepatopathy developed in a significant fraction of patients with long-term Fontan circulation and that transient elastography could be an easy and useful method to assess congestive hepatopathy in these patients.

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

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