Echogenic Mass Lesion within the Main Pulmonary Artery in a Neonate

Here we present a rare case of pulmonary arterial thrombosis associated with a ductus arteriosus aneurysm that caused severe pulmonary stenosis. A 5-day-old newborn was admitted to our hospital for the evaluation of an intracardiac mass-like lesion found after the detection of a cardiac murmur. Echocardiography and heart computed tomography revealed a mass-like lesion measuring 8.1 mm in diameter across the distal main pulmonary artery to the proximal left pulmonary artery resulting in localized severe stenosis of the left pulmonary artery. Left pulmonary artery angioplasty for surgical resection of the thrombus revealed that the mass was adherent to the proximal part of the left pulmonary artery anterior wall and extended to the ductus arteriosus. Histological examination of the mass showed an old thrombus with dystrophic calcification. Five months after surgery, follow-up echocardiography showed that the left pulmonary artery peak pressure gradient had decreased but the proximal left pulmonary artery stenosis remained. Cardiac catheterization and balloon angioplasty suc cessfully relieved the pulmonary stenosis.

A Pilot Study about Possible Gluten Sensitivity in Korean Urticaria Patients

No abstract available.

A Case of Pediatric Subcorneal Pustular Dermatosis / 대한피부과학회지

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson's disease, is a rare pustular eruption that occurs mainly in middle-aged women and rarely during childhood. Clinically, the pustules are distributed on the trunk and proximal region of the limbs, and cultures of the pustules consistently do not reveal bacterial growth. Histopathology shows subcorneal pustules containing polymorphonuclear leukocytes and chronic progression. SPD is a rare condition, especially in children, and only one case has been reported in Korea. Here, we report a case of 4-year-old girl as an educational case for the diagnosis and treatment of pediatric SPD.

A Case of Linear Syringocystadenoma Papilliferum on the Inguinal Area / 대한피부과학회지

No abstract available.

Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries

BACKGROUND AND OBJECTIVES: The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure. SUBJECTS AND METHODS: We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4). RESULTS: Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation. CONCLUSION: Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.

Surgery for Partial Anomalous Pulmonary Venous Connections: Modification of the Warden Procedure with a Right Atrial Appendage Flap

BACKGROUND: Surgical repair of a partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) may be complicated by sinus node dysfunction or SVC obstruction. We modified the Warden procedure by using a right atrial auricular flap to decrease the occurrence of these complications. METHODS: Between February 2005 and July 2012, 10 consecutive patients underwent a modified Warden procedure to correct PAPVC. The median patient age was 5.7 years. Eight patients (80%) had an atrial septal defect. To surgically correct the PAPVC, we made a U-shaped incision on the right atrial appendage and sutured the flap to the posterior wall of the SVC. The anterior wall was reconstructed with various patch materials. RESULTS: No early or late deaths occurred, nor did any patient require early or late reoperation for SVC or pulmonary venous obstruction. No new arrhythmias appeared during follow-up, which was complete in all patients (mean, 29.5 months). CONCLUSION: Our modification of the Warden procedure seems to be effective and safe. This technique may lower the risk of SVC obstruction, pulmonary venous obstruction, and sinus dysfunction.

Percutaneous Closure of the Acquired Gerbode Shunt Using the Amplatzer Duct Occluder in a 3-Month Old Patient

The Gerbode shunt, known as the left ventricle to the right atrial communication, is a rather rare finding, following surgical closure of septal defects. Even though the surgical closure is accepted as a treatment of choice, we report a successful percutaneous transcatheter closure of the Gerbode shunt in a 3-months old baby who weighed 3 kilograms.

An Adolescent Patient with Coarctation of Aorta Treated with Self-Expandable Nitinol Stent

Transcatheter treatment of aortic coarctation, with balloon angioplasty or stent implantation, is now an acceptable alternative to surgical repair. However these procedures may result in complications, such as vascular wall injury and re-stenosis of the lesion. A nitinol self-expandable stent, when deployed at the coarctation site, produces low constant radial force, which may result in a gradual widening of the stenotic lesion leaving less tissue injury ('stretching rather than tearing'). For an adolescent with a native aortic coarctation, a self-expandable stent of 20 mm diameter was inserted at the discrete stenotic lesion of 5 mm diameter without previous balloon dilatation procedure. No further balloon dilatation was done immediately after the stent insertion. With the self-expandable stent only, the stenosis of the lesion was partially relieved immediately after the stent deployment. Over several months after the stent insertion, gradual further widening of the stent waist to an acceptable dimension was observed.

The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder / 소아과

PURPOSE: Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO). METHODS: We retrospectively reviewed the medical records of 5 patients who underwent hybrid perventricular closure of MVSDs with ADOs, from March 2006 to May 2011. The median patient age at the time of the procedure was 12 months (range, 25 days to 25 months), and the median body weight was 9.1 kg (range, 4.3 to 15 kg). Two patients had multiple ventricular septal defects (VSDs; additional perimembranous VSD in 1 patient and multiple MVSDs in the other) and 3 patients had associated cardiac anomalies; complete transposition of the great arteries in 1 patient and an atrial septal defect in 2 patients. All the procedures were performed on beating hearts, exception in 1 case. The ADO selected for the aortic side was at least 1 to 2 mm larger than the largest VSD in the left ventricle side. RESULTS: The procedure was successful in all patients and each device was well positioned. During the median follow-up of 2.4 years, a small residual VSD was noted in 2 patients who had multiple VSDs and no leakage was seen in the other 3 patients. CONCLUSION: Perventricular closure of MVSD with an ADO is a good option for patients with apical MVSD. However, careful manipulation is important, especially in the case of small infants.

The Recent Surgical Result of Total Anomalous Pulmonary Venous Return

BACKGROUND AND OBJECTIVES: Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute. SUBJECTS AND METHODS: A total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction. RESULTS: The study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery. CONCLUSION: Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.

Ambulatory Blood Pressure Monitoring and Blood Pressure Load in Obese Children

BACKGROUND AND OBJECTIVES: This study was aimed at evaluating the significance of blood pressure (BP) load in ambulatory blood pressure monitoring (ABPM) in obese children and adolescents. SUBJECTS AND METHODS: ABPM was conducted for 60 selected patients who had visited Sunlin Hospital between January 2008 and August 2008. Patients were classified into 3 groups; an obese group whose body mass index (BMI) was > the 95th percentile, an overweight group whose BMI was > the 85th percentile but less than the 95th percentile, and a normal group whose BMI was below the 85th percentile. Overall mean BP, day and night BP and BP load were measured by ABPM. RESULTS: Of the 60 patients, twenty-seven children belonged to the obese group, 9 and 24 to the overweight and the normal group, respectively. Among the three groups, the overall average systolic and diastolic BP, daytime diastolic BP, and systolic BP loads in daytime and nighttime were statistically different. Comparing the obese group with the normal group, systolic BP loads in daytime and nighttime in the obese group were significantly higher than those in the normal group. Also, the obese group had more patients whose BP loads were over 25% greater than the normal group while the difference in the number of patients with overall hypertension was not significantly different. CONCLUSION: Assessment of children's BP through assessment of BP load is a more detailed and precise tool than assessment through mean BP using ABPM and BP can be better controlled using measurement of BP load.

The utility of ambulatory blood pressure monitoring in obese children / 소아과

PURPOSE: Obesity in children and adolescence is highly correlated with adult obesity, which can provoke hypertension. Therefore, it is important to evaluate the blood pressure of obese children regularly. In this study, the results of ambulatory blood pressure monitoring (ABPM) in obese children and adolescents were evaluated. METHODS: ABPM was conducted for selected patients who visited Handong University Sunlin Hospital from Feb. 1, 2006 to Dec. 1, 2007. The patients were classified into 3 groups: group 1 was normal, group 2 had normal casual blood pressure and a body mass index over the 95th percentile, and group 3 had high casual blood pressure over 120/80 mm Hg and a body mass index over the 95th percentile. Systolic and diastolic 24-hour blood pressure was measured, including both day and night. RESULTS: There were 49 patients in the study. The results showed a significant difference for average systolic blood pressure between the three groups (105.1+/-4.7, 111.0+/-7.1, 117.8+/-6.6 mmHg, P<0.001), but for average diastolic blood pressure only between groups 1 and 3 (69.1+/-5.3, 77.9+/-6.3 mmHg, P=0.001). In the daytime, only groups 1 and 3 showed a statistically significant difference for systolic and diastolic blood pressure. During the night the systolic pressure of group 3 was significantly higher than the other groups, but the diastolic pressure of group 3 was only higher than that group 1. No statistical difference was found in night dips among the groups. CONCLUSION: Ambulatory blood pressure monitoring in children and adolescents showed statistically higher blood pressure in obese patients with high casual blood pressure.

Antibiotic Sensitivity Pattern of Pathogens from Children with UTI

PURPOSE: We studied the degree of changes in antibiotic sensitivity toward causative organisms, prevalence and clinical manifestations of extended-spectrum beta-lactamase(ESBL)- producers of urinary tract infection(UTI) for a period of three years. This serves to provide useful information in selecting adequate drugs for the treatment of UTI. METHODS: We recruited 137 patients who grew more than 105 CFU/mL in their urine culture among 250 patients who visited and were admitted to Handong University's Sunlin Hospital for UTI treatment from January 2003 to December 2005. We retrospectively analyzed the data from the medical records. RESULTS: The common pathogenic organisms were Escherichia coli(65.0%), Klepsiella pneumoniae(14.0%), Enterococcus faecalis(5.8%) and Proteus vulgaris(2.9%) in consecutive order. The prevalence of ESBL-producers among isolated E. coli and K. pneumoniae was 4.5%(4 cases) and 14.3%(2 cases), respectively. The antibiotic sensitivity rates of E. coli were relatively high to amikacin(100%), imipenem(100%), ceftriaxone(95.5%) and tobramycin(91.4%) while relatively low to TMP/SMZ(55.4%), ampicillin/sulbactam(29.4%) and ampicillin (24.2%). CONCLUSION: The use of ampicillin, ampicillin/sulbactam and TMP/SMZ, which have been the first choices in the treatment of UTI, should be reconsidered due to the low sensitivity rates towards these antibiotics. Due to the high incidence and antibiotic tolerance of ESBL that might have risen from the development of new antibiotics and increased antibiotic use, it is necessary to consider changing the standard antibiotics that have been used in the treatment of UTI.

Successful Treatment of Tachycardia-induced Cardiomyopathy with Radiofrequency Catheter Ablation

Tachycardia-induced cardiomyopathy is caused by chronic tachyarrhythmias. It is characterized by ventricular systolic dysfunction and dilatation and by clinical manifestations of heart failure. We experienced three children with tachycardia-induced cardiomyopathy. Two with ectopic atrial tachycardia and one with left ventricular tachycardia were treated successfully by radiofrequency catheter ablation. The clinical and echocardiographic features of cardiomyopathy induced by tachyarrhythmia were reversible after rhythm control. Tachycardia-induced cardiomyopathy should be considered in patient with unexplained systolic dysfunction and any form of tachyarrhythmia as a reversible etiology. Radiofrequency catheter ablation can be a safe and effective treatment in patients with tachyarrhythmia which is complicated by ventricular dysfunction.

Study of Neonatal Cardiac Catheterization for Over the Last 10 Years

PURPOSE: The neonatal cardiac catheterization and angiogram for transcatheter therapy are still essential methods in congenital heart disease, so we reviewed our experience with neonatal cardiac catheterization over 10 years at a single institution. METHODS: A retrospective review of all 139 neonatal catheterizations from January 1991 to December 2000 at Sejong Heart Institution was performed. The purpose of the catheterizations, ages, body weights and the complications by the exam was surveyed. RESULTS:The mean age of our 139 patients was 14.9 days and the mean body weight was 3.3 kg. As for the diagnosis : TGA in 49 patients, PAIVS in 26 patients, DORV in 14 patients, PS in 14 patients, PAVSD in 11 patients. For the cardiac catheterization, general anesthesia was performed in 65% of total and fluoroscopy time was 20.1+/-14.5 minutes during cardiac catheterization. In the interventional cardiac catheterization which was 75% of the total, the age and body weight were statistically the same but the irradiation time was longer than the diagnostic catheterization(P=0.001). There were 48 cases of atrial septostomy, 16 cases of balloon pulmonary valvuloplasty and 25 cases of transcatheter pulmonary valvotomy. Complications of cardiac catheterization were found in 16.3%, but there was no difference between interventional catheterization and diagnostic catheterization. CONCLUSION: Therapeutic cardiac catheterization in neonates is a relatively safe and effective method in congenital heart disease.

The Influence of the Preoperative Chronic Cyanosis on the Cardiac Troponin I in the Pediatric Cardiac Operation

PURPOSE: The purpose of this study was to evaluate the perioperative myocardial damage in pediatric congenital heart disease with the cardiac specific protein of cardiac troponin I(cTpn-I). METHODS: All 25 pediatric patients who were diagnosed with tetralogy of Fallot or double outlet right ventricle were classified as group A(acyanotic, SaO2 >90%), group B(mildly cyanotic, SaO2 >80-90%) and group C(moderately cyanotic, SaO2 <80%). The control group D was consisted of 10 patients with ventricular septal defects who were operated in the same period. We measured preoperative hemoglobin, preoperative and postoperative(24 and 72 hour) arterial oxygen saturation, cTpn-I and creatine kinase(CK-MB). RESULTS: Total 25 patients were subdivided into 6 of group A, 12 of group B and 7 of group C. The concentrations of preoperative cTpn-I were 0.23+/-0.12 ng/mL in group A, 0.25+/-0.12 ng/mL in group B, 0.26+/-0.13 ng/mL in group C. And the concentrations of cTpn-I in postoperative 24 hour were 10.04+/-5.28 ng/mL in group A, 12.50+/-6.86 ng/mL in group B, 12.55+/-9.90 ng/mL in group C. Which revealed cTpn-I in group C was higher than that of the another less cyanotic groups. In addition, the concentration of cTpn-I of group C in postoperative 72 hour was higher than any other groups. The concentration of cTpn-I in postoperative 72 hour was statistically correlated with that in postoperative 24 hour and preoperative arterial oxygen saturation(P=0.001). CONCLUSION: Preoperative chronic cyanosis can influence on the postoperative concentration of cTpn-I in pediatric cardiac patients, which means impairment on the postoperative myocardial recovery.

A Case of Ascites by Pseudomembranous Colitis, Initially Diagnosed Malignant Asites / 대한소화기내시경학회지

Pseudomembranous colitis (PMC) is mostly related with the antibiotics and it presents with diarrhea, abdominal pain, fever, hypoalbuminemia and hypovolemia. In the clinical course of pseudomembranous colitis (PMC), ascites is a rare presentation, and high elevation of carcinoembryonic antigen (CEA) associated with PMC is also a very rare presentation. We experienced a case taken cephalosporin group antibiotics for six weeks and presented with fever, abdominal pain, severe diarrhea, and massive ascites. During evaluation, we found low serum-ascites albumin gradient and high level of CEA in both ascites and plasma. With the impression of hidden malignancy, the special studies were done, but PMC was only found without malignancy. After vancomycin therapy, all symptoms were relieved and CEA level declined.

Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography

PURPOSE: Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. METHODS: This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations including echocardiography were done, catheterization/angiography was performed the next day and the results were compared with those of echocardiography. The study population consisted of 9 patients, diagnosed as PAVSD in our hospital, from Jan. 1995 to Dec. 1997. RESULTS: Pulmonary blood was supplied via ductus arteriosus, in 3 patients(group 1), and in the other 6 patients, via major aortopulmonary collateral arteries(MAPCA)(group 2). The characteristic findings of group 2 were cardiac murmur heard in the back area(3 cases), and abnormal pulmonary arborization pattern(2 cases). The echocardiographic diagnosis of source of pulmonary blood flow was correct in all cases except one case-who had had two previous shunt surgeries and additional MAPCAs. In group 2, the presence of pulmonary confluence was predicted in 3 out of 4 cases. The mean number of MAPCAs found by echocardiography was 2.3+/-1.2 and by angiography, 3.5+/-1.4. CONCLUSION: In evaluation of PAVSD patients, near complete characteristics of pulmonary blood supply were identified by echocardiogram. Using it, practitioners can make decisions for shunt surgery or the need for further evaluations, including cardiac catheterization which can be more easily executed with previous echocardiographic data.

A Case of Streptococcal Toxic Shock Like Syndrome with Pleural Effusion

Streptococcal toxic shock like syndrome is a rapidly progressive and a fatal disease like staphylococcal toxic shock syndrome. In spite of the wide expansion and development of potent antibiotics, streptococcal infection still threatens human being. Recently we experienced a patient with toxic shock like syndrome who was suffered from fever and neck pain with rapid progression to hypotension and multiorgan failure. Streptococcus pyogenes was proven in his throat culture which was done on admission. Pleural effusion and suspicious carditis with aortic and mitral valve regurgitation were examined on the third hospital day. He was treated successfully with antibiotics but we wanted to emphasize potentially fatal streptococcal toxic shock like syndrome in pediatric patients.

Catecholaminergic Polymorphic Ventricular Tachycardia in Children

PURPOSE: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is the disease entity of adrenergic dependent, potentially lethal tachyarrhythmia in a child with no structural heart disease, which manifest itself as a syncope or sudden death. The objective of this study is to present our experiences on this important, although rare, disease. METHODS: Retrospective analysis of 5 patients with episodes of syncope related to exercise, who were referred to our hospital from January 1985 to December 1998. RESULTS: All patients were male and the mean age at the time of the first syncopal episode was 5.1+/-3.2 years (range 1.3 to 10 years). There were no structural cardiac abnormalities in clinical and laboratory evaluations. In all, polymorphic ventricular tachycardia showing the characteristic pattern of CPVT in which, as the heart became stimulated adrenergically, isolated ventricular premature beats appeared, increased with rate, became polymorphic, finally formed burst with bidirectional salvoes and disappeared in resting state was induced during exercise test and/or isoproterenol infusion test. During the mean follow up period of 3.75+/-3.1 years (range 1 month to 7.3 years), one died suddenly. In this case, low dose of beta-blocker was administered because of associated sinus bradycardia resulting in incomplete control of the syncopal episodes. The other 4 cases were alive and asymptomatic by means of adequate modification of beta-blocker dosage and method of administration. CONCLUSIONS: This study emphasizes that CPVT is an important, although rare, cause of exercise related syncope in children and can be diagnosed by means of exercise test and/or isoproterenol infusion. beta-blockers were very effective in all cases, even though increasing amount of beta-blocker was frequently necessary to control ventricular arrhythmia in some cases.