The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute / 소아과

PURPOSE: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. METHODS: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1–4 years). Medical records were reviewed retrospectively. RESULTS: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%–70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months. CONCLUSION: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

BACKGROUND AND OBJECTIVES: Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. SUBJECTS AND METHODS: The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. RESULTS: The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. CONCLUSIONS: Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms.

Neonatal Outcomes of Premature Infants Who Were Delivered from Mother with Hypertensive Disorders of Pregnancy and Effects of Antihypertensive Drugs and MgSO4 / 대한주산의학회잡지

PURPOSE: Hypertensive disorders of pregnancy increase morbidity and mortality of fetus and neonates. Recently some studies revealed that antihypertensive agents affected the neonatal outcomes. The aim of this study was to investigate the prognosis of preterm infants delivered from the mothers with hypertensive disorders who were treated with antihypertensive agents and magnesium sulfate. METHODS: This retrospective study was conducted on preterm infants who were delivered from normotensive mother (control, n=436) and antihypertensive drugs +/- magnesium sulfate treated mother (study, n=150) between January 2009 and December 2013. Study group were divided into two groups based on whether they received antihypertensive drugs only (n=110) and additional magnesium sulfate (n=40). We compared the characteristics of mothers and neonatal outcomes. RESULTS: Study group had shorter gestational age (32.2+/-3.5 weeks vs. 33.7+/-3.0 weeks, P=0.000) and lower birth weight (1,810.5 +/- 689.2 g, 2,212.1 +/- 604.9 g, P=0.000), and higher rate of small for gestational age infants (22% vs 11%, P=0.000). One minute and 5 minutes Apgar score were lower, but duration of hospital days, oxygen supplement and mechanical ventilation were longer in study group. Respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus, retinopathy of prematurity, intraventricular hemorrhage occurred more in study group than control. The group treated with magnesium sulfate together with antihypertensive agent had lower 1 minute and 5 minutes Apgar score than the group taken antihypertensive agent only. CONCLUSIONS: Mothers with hypertensive disorders have increased the risk of preterm delivery, low birth weight, and high neonatal morbidity rate. Therefore it is important to predict and manage possible complication. Moreover, if magnesium sulfate is taken, careful neonatal monitoring is needed because of possible low Apgar score.

Risk factors of allergic sensitization in preschool children with chronic cough

PURPOSE: Chronic cough is one of the major symptoms of asthma and allergic sensitization and may appear prior to the onset of asthma. The object of this study was to investigate the risk of allergic sensitization in preschool children with chronic cough. METHODS: We reviewed the medical records of 99 preschool children presenting with chronic cough but not with allergic rhinitis, atopic dermatitis, recurrent wheezing, or lower respiratory tract infection between November 2011 and July 2013. RESULTS: Fifty-four children (55%) were sensitized at least one of the following inhalant allergens: Dermatophagoides pteronyssinus, Dermatophagoides farinae, cockroach, Alternaria alternata, dog dander, and cat epithelium. Children with allergic sensitization had a higher blood total IgE levels transformed by common logarithm (1.9+/-0.6 IU/mL vs. 1.3+/-0.5 IU/mL, P<0.001) and eosinophils (3.7%+/-2.5% vs. 2.7%+/-2.0%, P=0.043), more frequent parental history of allergy (68% vs. 48%, P=0.044) and less frequent history of breast milk feeding (68% vs. 86%, P=0.041) than those without. CONCLUSION: These results suggest that the ratio of allergic sensitization may be 50% or more in preschool children with chronic cough and that parental history of allergy and formula milk feeding may be associated with allergic sensitization.

A Case of Herpes Simplex Virus Type 2 Encephalitis of a Newborn Delivered by a Mother without Prenatal Screening / 대한주산의학회잡지

Neonatal herpes simplex virus (HSV) encephalitis is a rare disease nowadays because of prenatal screening test and management. It shows progressive central nervous system manifestations affecting predominantly temporal and frontal lobes. Early diagnosis of HSV encephalitis is important since even with the early initiation of high-dose intravenous acyclovir therapy, it results in serious morbidity among survivors. A 14-day-old neonate with fever and poor oral intake was admitted via emergency department. The next day she had seizures and the brain was damaged with permanent sequelae despite of early administration of intravenous acyclovir on day 2 of admission. We report a serious case of HSV encephalitis diagnosed as type 2 HSV by polymerase chain reaction and culture of a newborn without proper prenatal screening test.

Prevalence of Voiding Dysfunction and Constipation in Children with Attention Deficit Hyperactivity Disorder

PURPOSE: Attention deficit hyperactivity disorder (ADHD) has been associated with impairments in frontal inhibitory function and the catecholaminergic system. ADHD is diagnosed in 3-5% of children. Children with ADHD seem develop various forms of urinary problems such as nocturnal enuresis, dysfunctional voiding, and diurnal incontinence. However, no data exist to confirm the presence of these problems in Korean children with ADHD. We investigated the clinical findings of voiding dysfunction in children with ADHD. METHODS: Between October 2009 and March 2011, a total of 63 children (33 with ADHD, 30 with an upper respiratory infection, as a control group) were enrolled. ADHD was diagnosed using the diagnostic and statistical manual of mental disorders (DSM)-IV criteria. A comprehensive survey of voiding and defecation was administered. RESULTS: The patient group included 28 boys and 5 girls; the control group comprised 20 boys and 10 girls. The mean age was 9.09+/-2.8 years in the ADHD group and 8.58+/-3.1 years in the control group. Children with ADHD had a statistically significantly higher incidence of urgency (P=0.017), urge incontinence (P=0.033), and constipation (P=0.045). There was no significant difference in the incidence of straining, intermittency, holding maneuvers, or nocturnal enuresis. CONCLUSION: Children with ADHD in Korea have significantly higher rates of urgency, urge incontinence, and constipation than those without ADHD.

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea / 소아과

PURPOSE: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. METHODS: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. RESULTS: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5+/-5.4 years (range, 2 to 22 years). The follow-up duration was 28.9+/-20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. CONCLUSION: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

6-Thioguanine-Induced Hepatic Sinusoidal Obstruction Syndrome in a Leukemic Child with TPMT Heterozygote / 임상소아혈액종양

Hepatic sinusoidal obstruction syndrome (hSOS) can be developed as a common complication after hematopoietic stem cell transplantation (HSCT), and rarely after 6-thioguanine-based chemotherapy without HSCT. A four-year-old boy with heterozygotic polymorphism for thiopurine methyltransferase (TPMT) developed hSOS after he received chemotherapy with cytarabine, cyclophosphamide, intrathecal methotrexate and 6-thioguanine (6-TG) as reconsolidation chemotherapy of acute lymphoblastic leukemia (ALL). He was treated with defibrotide, N-acetylcysteine, urusodeoxycholic acid, glutathione, and supportive care. He recovered completely in nine days without long-term complication, and completed chemotherapy with 6-mercaptopurine without severe complication. We report a case of hSOS developed in an ALL patient with TPMT heterozygote after 6-TG based reconsolidation chemotherapy.

6-Thioguanine-Induced Hepatic Sinusoidal Obstruction Syndrome in a Leukemic Child with TPMT Heterozygote / 임상소아혈액종양

Hepatic sinusoidal obstruction syndrome (hSOS) can be developed as a common complication after hematopoietic stem cell transplantation (HSCT), and rarely after 6-thioguanine-based chemotherapy without HSCT. A four-year-old boy with heterozygotic polymorphism for thiopurine methyltransferase (TPMT) developed hSOS after he received chemotherapy with cytarabine, cyclophosphamide, intrathecal methotrexate and 6-thioguanine (6-TG) as reconsolidation chemotherapy of acute lymphoblastic leukemia (ALL). He was treated with defibrotide, N-acetylcysteine, urusodeoxycholic acid, glutathione, and supportive care. He recovered completely in nine days without long-term complication, and completed chemotherapy with 6-mercaptopurine without severe complication. We report a case of hSOS developed in an ALL patient with TPMT heterozygote after 6-TG based reconsolidation chemotherapy.

Aortic valve replacement surgery for a case of infantile Takayasu arteritis / 소아과

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.

Current Characteristics of Infective Endocarditis with Congenital Heart Disease: A Retrospective Survey of 121 Cases between 1985 and 2006

BACKGROUND AND OBJECTIVES: The relative proportion of infective endocarditis (IE) with congenital heart disease (CHD) has increased because of improved survival with CHD. This may affect the current profile of IE with CHD. The aim of this study is to assess the changing profiles of IE with CHD. SUBJECTS AND METHODS: All cases diagnosed from January 1985 to May 2006 were retrospectively reviewed and were analyzed according to the diagnosed period (period I-prior to 1995, period II-after 1996). Duke criteria were used for the definition of diagnosis. RESULTS: As a whole, 121 episodes occurred (63 episodes in period I and 58 episodes in period II). Although the mean age was similar for patients diagnosed in the two periods, the proportion of younger age patients was larger during period II. During period II, the number of non-surgical ventricular septal defect (VSD) cases decreased and the number of Rastelli procedure cases increased. Negative blood cultures were similar in patients for both periods. S. viridans was the most common causative organism in patients during both periods. Post-surgical cases and IE after a dental procedure increased during period II. The in-hospital mortality rate, total complication rate, and proportion of IE requiring early surgery were not different for patients in either period. However, the interval to early surgery was shorter for patients during period II (period I 35+/-21.1 days, period II 22+/-16.5 days, p=0.041). CONCLUSION: Our study indicates that IE in CHD is still a major problem with similar clinical features exhibited during the two different periods. The presence of non-surgical VSD as a major underlying defect indicates that one needs to pay more attention to the education of the population at risk.

A clinical analysis of juvenile dermatomyositis; focus on clinical manifestations at diagnosis / 소아과

PURPOSE: Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. METHODS: Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. RESULTS: Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. CONCLUSION: This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

Pulmonary Atresia with Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries: Management Strategy at Our Hospital and the Results

BACKGROUND AND OBJECTIVES: Based on our previous studies on pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), a management strategy for this disease was formulated at our hospital in 1999. To evaluate this strategy, it was applied to the new patients and their outcomes were measured. SUBJECTS AND METHODS: The subjects were a cohort of newly diagnosed patients who were without any prior interventions and these patients were treated at our hospital from January 2000 to December 2003. The management strategy focused on promoting the growth of the pulmonary arterial confluence (PAC), if present, by performing a right ventricle-pulmonary artery conduit operation (RV-PA conduit). If the PAC was large or absent, then the management plan was discussed among the surgeons after performing an exhaustive work-up. RESULTS: Seventeen patients were enrolled and their age at the time of diagnosis ranged from 8 days to 34 months with a median age of 3 months. The initial surgical method varied from one-stage total correction (4 cases) to a conduit operation with or without additional MAPCAs procedures (13 cases). All but three of the patients survived their initial surgery. There were two late deaths. In addition to the 3 survivors from one-stage total correction, 4 patients underwent a staged total corrective operation. The 5 remaining patients are still a subtotal correction state or a palliated state. CONCLUSION: When a large PAC is present, one-stage total correction is the best surgical option. However, when the PAC is small, then RV-PA conduit without any vascular procedures may be a good alternative. This staged approach makes a larger PAC, which enables surgeons to create a much easier vascular anastomosis later on.