A retrospective study of changes in skin cancercharacteristics over 11 years

Background@#The incidence of skin cancer, which is primarily caused by exposure to ultravioletradiation, has steadily increased in recent years. The authors of the present study sought to investigatechanges in the epidemiology of skin cancer by conducting a retrospective review of patientsdiagnosed with skin cancer who received related care at a single medical institution. @*Methods@#The present study included patients who were diagnosed with skin cancer and receivedtreatment at Gyeongsang National University Hospital from 2008 to 2018. The site andtype of skin cancer, the number of patients with skin cancer each year, the sex and sex ratio of thepatients, and changes in patients’ age at first diagnosis were examined through retrospectivechart reviews. @*Results@#The number of patients with skin cancer significantly increased, but statistically significantchanges were not found in patients’ sex, skin cancer sites, or the types of skin cancer. However,patients’ age at the first diagnosis of skin cancer showed a statistically significant decreasestarting in 2015. @*Conclusion@#In this study, the number of patients with skin cancer increased over time. However,patients’ age at first diagnosis has decreased since 2015. Therefore, younger patients should takecare to prevent skin cancer, and further research on the causes of skin cancer in younger patientsis needed.

Serum levels of carbohydrate antigen 125 in combination with N-terminal pro-brain natriuretic peptide in patients with acute decompensated heart failure

BACKGROUND/AIMS@#Carbohydrate antigen 125 (CA-125) is an emerging prognostic biomarker for heart failure. We aimed to test the long-term prognostic value of CA-125 in combination with N-terminal pro-brain natriuretic peptide (NT-proBNP) in patients with acute decompensated heart failure (ADHF).@*METHODS@#This observational study included a total of 413 patients (64.1 ± 15.6 year-old, 214 men) with ADHF. All-cause mortality during the 2-year follow-up was investigated for the prognosis.@*RESULTS@#During the follow-up (mean follow-up, 591 ± 233 days), 109 deaths (26.0%) were recorded. In the multivariable analysis model, CA-125 was an independent factor associated with all-cause mortality (log CA-125: hazard ratio, 1.23; 95% confidence interval, 1.02 to 1.48; p = 0.030) together with age, sex, New York Heart Association class, β-blocker, and NT-proBNP. The Kaplan-Meier survival analysis demonstrated that the group with both low marker levels showed the best 2-year survival (87.9%) followed by the group with low NT-proBNP and high CA-125 (76.1%), high NT-proBNP and low CA-125 (64.7%) and high NT-proBNP and high CA-125 levels (54.3%) (p < 0.001). Addition of CA-125 in combination with NT-proBNP and established risk factors further increased the predictive power for mortality in patients with ADHF.@*CONCLUSIONS@#CA-125 was an independent factor associated with all-cause mortality in patients with ADHF. Combination of CA-125 with NT-proBNP significantly improved the prediction of mortality in patients with ADHF.

Changes in Strain Pattern and Exercise Capacity after Transcatheter Closure of Atrial Septal Defects

BACKGROUND AND OBJECTIVES: Assessment of left ventricle (LV) function by using strain and strain rate is popular in the clinical setting. However, the use of these echocardiographic tools in assessing right ventricle (RV) failure, and the manner in which they both reflect the functional capacity of the patient, remains poorly understood. This study aimed to investigate the change in exercise capacity and strain between before and (1 month) after the transcatheter closure of atrial septal defects (ASDs). SUBJECTS AND METHODS: Thirty patients who underwent transcatheter closure of ASD between May 2014 and June 2015 at the Division of Pediatric Cardiology, Severance Cardiovascular Hospital, were enrolled. We compared and analyzed the results of the following examinations, before and (1 month) after the procedure: echocardiography, cardiopulmonary exercise test (CPET), and N-terminal pro-brain natriuretic peptide level. RESULTS: There were no mortalities, and the male-to-female ratio was 1:2. The mean defect size was 22.3±4.9 mm; the mean Qp/Qs ratio, 2.1±0.5; and the mean device size, 22.3±4.9 mm. Changes in global RV longitudinal (GRVL) strain and LV torsion were measured echocardiographically. Exercise capacity improved from 7.7±1.2 to 8.7±1.8 metabolic equivalents (p=0.001). These findings correlated to the change in GRVL strain (p=0.03). CONCLUSION: The average exercise capacity increased after device closure of ASD. The change in strain was evident on echocardiography, especially for GRVL strain and LV torsion. Further studies comparing CPET and strain in various patients may show increased exercise capacity in patients with improved RV function.

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.

Pulmonary Thromboembolism after Intravenous Immunoglobulin Therapy in Guillain-Barre Syndrome / 대한임상신경생리학회지

Intravenous immunoglobulin (IVIG) is a safe treatment to treat various neurological disorders, but fatal thrombotic events as rare complications have been reported. A 54-year-old woman with Guillain-Barre syndrome complained of dyspnea during IVIG treatment. She was finally diagnosed with pulmonary thromboembolism. To the best of our knowledge, this is the first case of pulmonary thromboembolism associated with IVIG treatment in a Korean patient with Guillain-Barre syndrome.

Transvenous Implantation of a DDDR Pacemaker in a Patient with Extracardiac Conduit Fontan Circulation / 대한내과학회지

As the survival rate of patients with complex congenital heart disease has improved and the number of adult patients with congenital heart disease has risen, arrhythmias and heart failure have become important issues in these patients. Cardiac implantable electronic devices, including pacemakers, are also on the rise. Transvenous implantation or epicardial pacemaker implantation is challenging in patients with complex congenital heart disease. Here we report a case in which a dual-chamber pacing, dual-chamber sensing, dual response and rate-adaptive (DDDR) pacemaker was implanted transvenously into a patient with congenital heart disease. A 34-year-old male with extracardiac conduit Fontan circulation complained of dizziness; an electrocardiogram revealed junctional bradycardia. We performed transvenous implantation of a DDDR pacemaker via trans-conduit puncture. In conclusion, transvenous implantation of a pacemaker is feasible in patients with extracardiac conduit Fontan circulation.

A Hypereosinophilic Syndrome with Cardiac Involvement from Thrombotic Stage to Fibrotic Stage

Cardiac involvement is a major cause of morbidity and mortality in hypereosinophilic syndrome (HES). It is classified into 3 stages by the degree of eosinophils-mediated heart injury; acute necrotic stage, thrombotic stage, and fibrotic stage. Nonetheless, definitive evidence that each patient passes sequentially through these stages is lacking. We present a case of 48-year-old male patient with dyspnea and peripheral edema who underwent valve replacement surgery due to severe mitral regurgitation. After the valve replacement, HES with cardiac involvement in the thrombotic stage was diagnosed. In the follow-up study, the patient progressed into fibrotic stage of HES.

Cardiopulmonary function and scoliosis severity in idiopathic scoliosis children / 소아과

PURPOSE: Idiopathic scoliosis is a structural lateral curvature of the spine of unknown etiology. The relationship between degree of spine curvature and cardiopulmonary function has not yet been investigated. The purpose of this study was to determine the association between scoliosis and cardiopulmonary characteristics. METHODS: Ninety children who underwent preoperative pulmonary or cardiac evaluation at a single spine institution over 41 months were included. They were divided into the thoracic-dominant scoliosis (group A, n=78) and lumbar-dominant scoliosis (group B, n=12) groups. Scoliosis severity was evaluated using the Cobb method. In each group, relationships between Cobb angles and cardiopulmonary markers such as forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC, left ventricular ejection fraction, pulmonary artery flow velocity, and tissue Doppler velocities (E/E', E'/A') were analyzed by correlation analysis linear regression. RESULTS: In group A, 72 patients (92.3%) underwent pulmonary function tests (PFTs), and 41 (52.6%) underwent echocardiography. In group B, 9 patients (75.0%) underwent PFT and 8 (66.7%) underwent echocardiography. Cobb angles showed a significant negative correlation with FVC and FEV1 in group A (both P<0.05), but no such correlation in group B, and a significant negative correlation with mitral E/A ratio (P<0.05) and tissue Doppler E'/A' (P<0.05) in group A, with a positive correlation with mitral E/A ratio (P<0.05) in group B. CONCLUSION: Pulmonary and cardiac function was significantly correlated with the degree of scoliosis in patients with thoracic-dominant scoliosis. Myocardial diastolic function might be impaired in patients with the most severe scoliosis.

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.

Case of Recurrent Ventricular Fibrillations with Osborn Wave Developed during Therapeutic Hypothermia

Therapeutic hypothermia (TH) has been used to protect neurological functions in cardiac arrest patient. Although Osborn wave is not pathognomonic of hypothermia, it is a well-known electrocardiogram finding of hypothermic patients. The cellular and ionic mechanisms of the Osborn wave have been suggested, and its relationship to tachyarrhythmias, such as ventricular tachycardia and ventricular fibrillation, is being explored. This case highlights the arrhythmogenic potential of Osborn wave and individual difference in response of TH.

Transvascular Implantation of an Implantable Cardioverter-Defibrillator in a Patient Who has Undergone One-and-a-Half Ventricle Repair

Implantable cardioverter-defibrillator (ICD) therapy is acknowledged as a valid treatment method for the effective prevention of sudden cardiac death, which is a major cause of mortality in adult congenital heart disease patients. But ICD implantation by the conventional transvascular approach is not always possible in patients who have undergone palliative surgery due to congenital and structural heart disease. Here, we report a case in which an ICD was transvascularly implanted in an arrhythmogenic right ventricular cardiomyopathy patient who had undergone a one-and-a-half ventricle repair.

A Case of Thymic Carcinoma Misdiagnosed as Epipericardial Fat Necrosis / 대한내과학회지

Epipericardial fat necrosis (EPFN) is an uncommon benign condition of unknown etiology. It presents as an unexplained acute severe pleuritic chest pain that is associated with the presence of a well-defined juxtacardiac mass usually located in or near the cardiophrenic angle. Although its typical clinical manifestations and chest computed tomography findings might lead to successful diagnosis of this rare disease, an unusual mass location such as the anterosuperior mediastinum should be considered and biopsies should be performed. We herein report a case of thymic carcinoma that was suspected initially to be EPFN.

Changes in Patient Characteristics of Infective Endocarditis with Congenital Heart Disease: 25 Years Experience in a Single Institution

BACKGROUND AND OBJECTIVES: The profile of infective endocarditis (IE) has changed and is now showing an increasing prevalence of IE among congenital heart disease (CHD) patients. We studied the change of clinical profiles of IE over the past 25 years in patients with CHD at a single institution. SUBJECTS AND METHODS: We reviewed medical records retrospectively for 325 patients diagnosed with IE between January 1, 1987, and March 31, 2012. We analyzed and compared the differences in patient characteristics and outcomes between 1987-2000 (group A) and 2001-2012 (group B). RESULTS: Over the 25-year period, 93 cases of IE in CHD patients were diagnosed (59 cases in group A and 34 cases in group B). Ventricular septal defect was the most common underlying cardiac disease observed during the entire period. The most common causative pathogen was Streptococcus in both groups. Group A contained 16 cases (27.1%) that had undergone cardiac surgery, whereas this number was 19 (55.8%) in group B. The number of patients who had undergone palliative care or surgery using prosthetic materials was higher among group B patients (p<0.001). Surgical procedures due to uncontrolled infection were performed in three cases in group A and 10 cases in group B. CONCLUSION: Infective endocarditis and CHD show a close correlation, and the profile of IE patients can change in line with an increase in the survival rate of patients with complex CHD and the improvement of surgical techniques. Ongoing reassessment and the systematic management of these patients is crucial in the prevention and treatment of IE.

Recurrent Syncope Episodes and Exercise Intolerance in Hypertrophic Cardiomyopathy Combined with Atrioventricular Conduction Disturbance

A 30-year-old female patient with known hypertrophic cardiomyopathy (HCMP) was admitted for recurrent syncope episodes. Electrocardiogram (ECG) showed 2 : 1 atrioventricular (AV) block. Stress echocardiography with bicycle showed high grade AV block at high stage of the exercise associated with exercise intolerance and dyspnea. Twenty-four hour ECG monitoring also revealed high grade AV block and 1 episode of non-sustained ventricular tachycardia. Implantable cardioverter/defibrillator-pacemaker (ICD-P) was inserted. After implantation of ICD-P, conduction disturbance and exercise intolerance were improved. AV block is a rare complication HCMP. There are just a few case reports that present symptoms caused by conduction disturbance in HCMP. This case describes repeated syncope episodes and exercise intolerance caused by conduction disturbance during exercise in HCMP patient. For evaluating the cause of syncope in HCMP, stress echocardiography can be helpful to understand the probable mechanism of syncope.

The Comparison between the Echocardiographic Data to the Cardiac Catheterization Data on the Diagnosis, Treatment, and Follow-Up in Patients Diagnosed as Pulmonary Valve Stenosis

BACKGROUND: Isolated pulmonary valve stenosis (PS) makes up 6-9% of all congenital heart defects among children. The initial gold standard for diagnosis, follow-up of PS is by echocardiography. However, the most accurate diagnosis still remains to be measurement of the pressure gradient through transcatheterization. The purpose of this study is to compare the difference between the echocardiographic data to the cardiac catheterization data on the diagnosis, treatment, and follow-up in patients diagnosed as PS, and to see what parameters should be closely monitored. METHODS: A total of 112 patients (Male : Female = 46 : 66) who underwent balloon pulmonary valvuloplasty (BPV) at Severance Cardiovascular Hospital, between December, 2002 to August, 2012 were retrospectively analyzed. The patients were all under 16 years of age and critical PS patients who underwent BPV were excluded from this study. RESULTS: The pre-BPV right ventricle (RV)-pulmonary artery (PA) systolic pressure gradient and post-BPV systolic pressure gradient showed statistically significant decrease. The pre-BPV RV-PA systolic pressure gradient and 3 month post-BPV systolic pressure gradient showed statistically significant decrease. The consistency between the echocardiographic data and cardiac catheterization data shows statistically significant consistency. The mean pressure gradient and systolic pressure gradient on the echocardiography shows high consistency when comparing with the cardiac catheterization data. CONCLUSION: Our study shows that BPV in PS is a safe and effective procedure in children and adolescent. The standard echocardiographic evaluation of PS, during diagnosis and follow-up, should include mean transpulmonic pressure gradient, as well as the peak systolic pressure gradient. The success of the procedure should be held off until at least 3 months, only if the patients do not show any symptoms.

Transcatheter Treatment of Patent Foramen Ovale Combined with Abnormal Drainage of Left Superior Vena Cava to Left Upper Pulmonary Vein

Patent foramen ovale (PFO) has been known to be the cause of transient ischemic attacks or stroke, and transcatheter device closure has been the treatment of choice for these defects. Combined defect of abnormal drainage of left superior vena cava (LSVC) to left superior pulmonary vein (LSPV) in PFO patients is an uncommon combination, and both can act as a pathway for paradoxical embolism. We report a successful closure of PFO, using Amplatzer(R) PFO occluder (St. Jude Medical, St. Paul, MN, USA) and persistent LSVC connected to LSPV using an Amplatzer(R) Vascular Plug II (St. Jude Medical, St. Paul, MN, USA). Because this combined anomaly of PFO and persistent LSVC can be treated by a single transcatheter intervention, if clinically suspected, a complete evaluation for this anomaly should be considered.

QTc Prolongation after Ventricular Septal Defect Repair in Infants

BACKGROUND AND OBJECTIVES: Prolonging of the corrected QT interval (QTc) has been reported after cardiac surgery in some studies. However, there have not been many studies on infant open cardiac surgery for ventricular septal defect (VSD) repair. This study was performed to define the changes in QTc and to find related post-surgery factors in this patient group. SUBJECTS AND METHODS: From 2008 to 2012, 154 infants underwent VSD repair at the Severance Cardiovascular Hospital. This study includes 105 of these cases. QTc was measured in these patients retrospectively. Demographic data and peri-procedural data, such as Aristotle score, cross-clamp time and bypass time, were analyzed. The exclusion criteria included multiple and small VSDs that underwent direct closure. RESULTS: Mean post-operative QTc was increased compared to the pre-operative measurements (from 413.6+/-2.3 to 444.9+/-2.5, p<0.001). In multiple linear regression, the comprehensive Aristotle score was associated with increasing QTc (p=0.047). The incidence of transient arrhythmia, such as atrial tachycardia, junctional ectopic tachycardia, premature atrial contraction, or premature ventricular contraction, was associated with QTc prolongation (p=0.005). Prolonged QTc was also associated with cross-clamp time (p=0.008) and low weight (p=0.042). Total length of stay at the intensive care unit and intubation time after surgery were not associated with QTc prolongation. CONCLUSION: Prolonged QTc could be seen after VSD repair in infants. This phenomenon was associated with peri-procedural factors such as the Aristotle score and cross-clamp time. Patients with QTc prolongation after cardiac surgery had an increased tendency towards arrhythmogenicity in the post-operative period.

Clinical Results of High-Dose Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation in Children with Advanced Stage Rhabdomyosarcoma

Regardless of improvement in cure of Rhabdomyosarcoma (RMS), the results in treatment of advanced stage of RMS in children are still dismal. Recently, high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (HDC/APBSCT) has been tried to manage the advanced high-risk RMS patients. We investigated the effectiveness of HDC/APBSCT by reviewing the clinical records of high-risk pediatric RMS patients in single institute database. Over twenty years, 37 patients were diagnosed as RMS with high-risk at the time of first diagnosis. These patients were classified as two groups according to treatment method. The first group was HDC/APBSCT and the other was conventional multi-agent chemotherapy group. Differences of clinical results between the two groups were analyzed. The median age of patients was 5 yr, ranging from 6 months to 15 yr. The 5-yr event free survival rate (EFS) of all patients was 24.8% +/- 4.8%. HDC/APBSCT group and conventional multi-agent chemotherapy group were 41.3% +/- 17.8% and 16.7% +/- 7.6% for 5-yr EFS, respectively (P = 0.023). There was a significant difference in the result of HDC/APBSCT between complete remission or very good partial response group and poor response group (50% +/- 20.4% vs 37.5% +/- 28.6%, P = 0.018). HDC/APBSCT can be a promising treatment modality in high-risk RMS patients.

Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension / 소아과

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

Respiratory syncytial virus prevention in children with congenital heart disease: who and how? / 소아과

Respiratory syncytial virus (RSV) is a major cause of respiratory infection in children. Most of the pediatric population have RSV infection before the age of 2, and recurrent infections are common even within one season. Chronic lung disease, prematurity, along with congenital heart disease (CHD) are major risk factors in severe lower respiratory infection. In hemo-dynamically significant CHD patients with RSV infection, hospitalization is usually needed and the possibility of treatment in intensive care unit and the use of mechanical ventilator support are known to increase. Therefore the prevention of RSV infection in CHD patients is mandatory. The current standard for RSV prevention is immunoprophylaxis by palivizumab. Immunoprophylaxis is recommended monthly in hemodynamically significant CHD patients, up to 5 months. Motabizumab, a second generation drug and newly developing RSV vaccines are also expected to play a key role in RSV prevention in the future. The prophylaxis of RSV infection in CHD patients is cost-effective in both the medical aspect of the patients as well as the socio-economic aspect. Therefore an effort to promote prevention should be made by not only the family of the patients but also by the government.