• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.917-929
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• 2006

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.1
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• pp.139-145
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• 1999

Congenital Insensivity to Pain with Anhidrosis(CIPA) is rare autosomal recessive disorder which is known to be hereditary sensory and autonomic neuropathies(HSAN) type IV. It is predilection for male and observed in early infant. Its clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mild to moderate mental retardation. Its dental features include self-mutilation by nail peeling, which leads to gingival ulcer, tongue ulcer due to tongue biting, and enamel hypoplasia. Partial anodontia is often observed as well. Seventeen-months-old boy with CIPA was reported. This is the case in which the dental characteristics are described and the dental treatment of patient is discussed.

• Journal of Korean Academy of Nursing
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• v.47 no.2
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• pp.178-187
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• 2017

Purpose: The purposes of this study were to identify the relationships among perceived parental bonding, illness perception, and anxiety and to determine the influences of perceived parental bonding and illness perception on anxiety in adult patients with congenital heart diseases. Methods: In this study a descriptive correlational design with survey method was utilized. The participants were 143 adult patients with congenital heart disease being cared for in the cardiology out-patient clinic of A medical center. Data were collected using the Parental Bonding Instrument, Illness Perception Questionnaire Revised Scale, and Cardiac Anxiety Questionnaire Scale. Data were analyzed using descriptive statistics, independent t-test, one-way ANOVA, Pearson correlation analysis, and hierarchial regression analyses. Results: There showed significant positive relationships of anxiety with maternal overprotection, consequences, and personal control respectively. Among predictors, maternal overprotection (${\beta}=.45$), consequence (${\beta}=.26$), and personal control (${\beta}=-.03$) had statistically significant influence on anxiety. Conclusion: Nursing interventions to decrease maternal overprotection and negative consequence, and to enhance personal control are essential to decrease the anxiety of adult patients with congenital heart diseases.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.250-258
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• 1986

From Aug. 1984 to May 1986, 116 cases of cardiovascular surgery including 85 cases of open heart surgery were performed at Korea Veterans Hospital. There were 108 congenital anomalies and 8 acquired valvular heart diseases. Among 108 congenital malformations 92 operations were done for acyanotic group, and 16 operations for cyanotic group. Thirteen cases of open heart surgery for infants or small children under 10kg of body weight were performed, which occupied 15.3 percent of total open heart surgery done in the same period. Common congenital cardiovascular anomalies were ventricular septal defect [40.7%], patent ductus arteriosus [29.6%], tetralogy of Fallot [12.[%], atrial septal defect [11.1%], and pulmonary stenosis [1.9%] in order of frequency. Valve replacement using lonescu-Shiley or Carpentier-Edwards valve was performed for 8 cases of acquired mitral valve disease, and valve replacement using St. Jude valve was done for a case of patent ductus arteriosus with severe mitral insufficiency. There was no mortality in acyanotic congenital malformations and acquired valve lesions, and 5 cases of mortality in cyanotic congenital malformations. Overall mortality was 4.3 percent for total cases and 5.9 percent for 85 cases of open heart surgery.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.265-272
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• 1985

Congenital aneurysm of sinus of Valsalva is one of the rare congenital heart disease, which is usually asymptomatic until rupture. The aneurysm usually ruptures into a cardiac chamber and produces an aorto-intracardiac fistula. Ruptured aneurysm is a grave lesion in that it causes heart failure and subsequent death. If, however, it is discovered in its early stages and operated on properly, it can be corrected with considerable success. Form January 1975 through December 1984, 18 consecutive patients with congenital aneurysm of sinus of Valsalva underwent corrective surgery using total cardiopulmonary bypass in our department of Thoracic Surgery. 1. The incidence was about 0.9% of surgical cases of congenital heart disease during that period. 2. 13 were males and 5 females, with ages ranging 12 years to 52 years. 3. Associated anomalies were VSD in 14, infundibular PS in 1, aberrant muscle band in RVOT in 1, and secondary aortic insufficiency in 9. 4. 17 were suggested to arise from right coronary sinus and 1 from noncoronary sinus; Among 17, 12 ruptured into right ventricle, and one from noncoronary sinus into right atrium. 5. Surgical correction was performed by means of direct suture closure with combined pledget or patch graft after aneurysm resection, and associated lesions were also corrected simultaneously. 6. There was only one case of operative mortality, and all the other patients were relatively uneventful in their follow-up studies.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.60-64
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• 2008

Kabuki syndrome is characterized by peculiar facial features, developmental delay, and mental retardation. Congenital hepatic abnormalities in Kabuki syndrome patients have been sporadically reported in the literature and consist of extrahepatic biliary atresia, neonatal sclerosing cholangitis, and transient neonatal cholestasis. We report here a case of congenital hepatic fibrosis in a patient with Kabuki syndrome. To our knowledge, only one case of congenital hepatic fibrosis has been reported in the setting of Kabuki syndrome.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.659-672
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• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.308-311
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• 2003

Congenital chloride diarrhea is a serious autosomal recessive disease, and defect of intestinal electrolyte absorption that involves, specifically, $Cl^-/{HCO_3}^-$ exchange in the distal part of the ileum and colon. The clinical feature is dominated by profuse, watery diarrhea containing high concentrations of chloride(>90 mmol/L) and sodium. The chloride loss results in severe dehydration with a hypochloremic alkalosis. The molecular pathology involves an epithelial $Cl^-/{HCO_3}^-$ exchanger protein. Mucosal ion transport is affected to differing degrees and the severity of the disease may thus vary. Recently, a gene defect on chromosome 7 has been identified. However, there was a deficit in replacement of fluid and electrolyte, abdominal distension remained and the character of stools was watery. We report a case of congenital chloride diarrhea in a premature female who presented with watery diarrhea containing high concentrations of chloride and abdominal distension.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.584-587
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• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Archives of Craniofacial Surgery
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• v.9 no.1
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• pp.41-44
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• 2008

Cleft palate and congenital alveolar synechia is a rare syndrome. Only eight cases have been previously reported. It consists of a spectrum of facial anomalies always including cleft palate and congenital alveolar synechiae without other abnormalities. This report described an unusual case of congenital alveolar synechial band spanning posterior alveolar of the two jaws with cleft palate. Previously reported cases showed bilaterally or anteriorly located fibrous band. In our department, a new born revealed unilateral posterior synechia. Under brief intravenous sedation, synechium was divided using bipolar diathermy in the nursery at 3 days of age because of poor feeding. This division allowed full jaw opening after brief passive exercise. The patient is growing and maturing as expected with no complications. This patient is supposed to be the first reported case of isolated unilateral alveolar synechium combined with cleft palate in the worldwide.