• The Journal of the Korea Contents Association
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• v.20 no.9
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• pp.659-671
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• 2020

Severe congenital heart disease (CHD) is a chronic disease requiring continuous and holistic health management to improve patients' quality of life. This study explored the health management experiences of adolescents with severe CHDs in Korea. On the basis of in-depth interviews with nine youths, four categories and 12 subcategories were identified. The participants lacked a full understanding of their health status and the need for appropriate health management for their health status. Nonetheless, they practiced health management to some extent, in ways that were affected by heart function, self-identity, and social support. Although adolescents with severe CHDs were affected by physical limitations and the discriminatory gaze around them, they tried to achieve normalcy through managing information, seeking homogeneity with peers, and fostering positive acceptance about their disease. Regarding demands for health management programs, disease knowledge and mental health content were preferred, and the preferred delivery methods were self-help group camps and smartphone applications. Based on the results of this study, we suggest a health management intervention to promote a healthy transition to adulthood for adolescents with severe CHDs, and that policy-makers should consider measures that would enable a successful transition to adulthood in the future of youth with severe CHDs.

• Journal of the korean academy of Pediatric Dentistry
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• v.43 no.3
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• pp.313-319
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• 2016

Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The "Greek warrior helmet appearance" is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism. Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and cone-shaped and taurodontic teeth. This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia. This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.

• Pediatric Infection and Vaccine
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• v.23 no.3
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• pp.209-216
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• 2016

Purpose: This study aimed to evaluate and compare the characteristics of infective endocarditis (IE) between children and adults with congenital heart disease (CHD) at a single tertiary care center. Methods: In this retrospective medical record review, we extracted the demographic characteristics, diagnostic variables, and outcomes of patients diagnosed with IE and CHD between 2000 and 2016. Results: We identified a total of 14 pediatric patients (nine male; median age at diagnosis, 3 years). Of the 14 patients, six had a history of previous open heart surgery, while four had undergone tetralogy of Fallot repair, with transannular patch or Rastelli procedure. Among the 10 children with positive blood cultures, the most common isolated organism was Staphylococcus spp. (8/10, 80%). Eleven adult patients had IE and CHD. Among the adult patients, only four were diagnosed with CHD before IE, and ventricular septal defect was the most common CHD. The most common isolated organism was Streptococcus spp. (6/11, 55%). Compared with adult patients, pediatric patients had a higher incidence of previously diagnosed CHD (P=0.001), with Staphylococcus spp. as the causative organism (P=0.027). The median duration between the onset of symptoms and diagnosis of IE was 9 days in children and 42 days in adults (P=0.012). Conclusions: Significant differences with regard to the diagnosis and progress of IE were observed between children and adults. Age-adjusted and systematic reassessment may be necessary for the diagnosis and management of IE.

• Clinical and Experimental Pediatrics
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• v.55 no.7
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• pp.232-237
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• 2012

Purpose: With feasibility in the diagnoses of congenital heart disease (CHD) in the antenatal period, we suspect changes have occurred in its incidence. No data have been reported about the current incidence of simple forms of CHD in Korea. We have attempted to assess the recent incidence and characteristics of CHD in the neonatal care unit of a secondary referral medical center. Methods: Medical records of 497 neonatal care unit patients who underwent echocardiography in the past 5 years were reviewed. Preterm infants with patent ductus arteriosus and other transient, minimal lesions were excluded from this study. Results: Although the number of inpatients remained stable, the incidence of simple forms of CHD showed a gradual decrease over the 5-year study period; a markedly low incidence of complex forms was seen as well. CHD was observed in 3.7% full-term and 6.8% pre-term infants. CHD was observed in 152 infants weighing >2,500 g (3.5% of corresponding birth weight infants); 65 weighing 1,000 to 2,500 g (9.3%); and 6 weighing <1,000 g (8.0%). The incidence of CHD was higher in the pre-term group and the low birth weight group than in each corresponding subgroup (P<0.001); however, the incidence of complex CHD in full-term neonates was high. The number of patients with extracardiac structural anomalies has also shown a gradual decrease every year for the past 5 years. Conclusion: Findings from our study suggest that the recent incidence and disease pattern of CHD might have changed for both complex and simple forms of CHD in Korea.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.22 no.4
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• pp.449-453
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• 2000

Soft tissue expansion is widely used technique in oral & maxillofacial reconstruction and provide new method of reconstruction in posttraumatic alopecia, post burn, wide scar, congenital deformity, benign tumor, tattoo, etc. Expanded tissue flaps have the advantage of increased vascularity, proximity to the defect, and similarity of color and texture. They also preclude the need to advance flaps from distant sites. Tissue expansion can be used to form a well vascularized cavity to accomodate and nourish bone grafts. The following report describes the uses of tissue expanders by allowing bone grafting to correct both soft and bony defects of mandibular region

• Journal of Genetic Medicine
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• v.16 no.2
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• pp.71-75
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• 2019

Periventricular nodular heterotopia (PNH) is a malformation of cortical development in which normal neurons inappropriately cluster in periventricular areas. Patients with Mowat-Wilson syndrome (MWS) typically present with facial gestalt, complex neurologic problems (e.g., severe developmental delay with marked speech impairment and epilepsy), and multiple anomalies (e.g., Hirschsprung disease, urogenital anomalies, congenital heart defects, eye anomalies, and agenesis of the corpus callosum [CC]). MWS is mostly caused by haploinsufficiency of the gene encoding zinc-finger E-box-binding homeobox 2 (ZEB2) due to premature stops or large deletions. We present a case report of a 9-year-old girl with PNH, drug-responsive epilepsy, severe intellectual disability, and facial dysmorphisms only in whom we performed whole-exome sequencing and found a de novo heterozygous missense mutation (c.3134A>C; p.His1045Pro) of ZEB2 (NM_014795.3; NP_055610.1). This mild case of MWS caused by a rare novel missense mutation of ZEB2 represents the first report of MWS with isolated PNH.

• Journal of Chest Surgery
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• v.28 no.3
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• pp.237-246
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• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8 kg [mean weight, 4.0 1.4 kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1 $\pm$ 3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients [86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septum was resected whenever necessary. There were seven early deaths [16.8 % , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis, stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily. We concluded that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are reasonable.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.766-771
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• 2003

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. Truncus arteriosus is a rare congenital anomaly that has an unfavorable natural course. We report a successful surgical correction of truncus arteriosus in an 13-day-old premature infant with body weight of 1.5 kg and gestational age of 32 weeks. We used autologous untreated pericardial conduit without valve in right ventricular outflow reconstruction. The patients remains in good condition with normal body weight (50 percentile) and wide right ventricular outflow tract 20 months after the operation.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.174-181
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• 1985

From Jun. 1980 to Mar. 1984, 33 cases of atrial septal defect, secundum type, operated at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Keimyung University, were analyzed retrospectively. They were 24.3% of all congenital heart diseases operated in the same period. Among the 33 cases, 17 cases were males and 16 cases were females. Their ages were ranged from 4 to 42 years and cases of over 15 years of age were 60.6%. Main symptoms at admission were exertional dyspnea [69.7%], palpitation [63.6%] and frequent upper respiratory infections [51.5%], but 2 cases [6.1%] were asymptomatic. All 33 cases were operated under the direct vision with cardiopulmonary bypass. All cases were secundum type defect of atrial septal defect and single defect were in 29 cases [90.6%], and oval type defect were in 31 cases [96.9%]. In 7 cases [21.9%], other lesions of cardiovascular system were associated, and the most common lesion was pulmonic valvular stenosis [4 cases; 12.5%]. The defects were closed directly in 27 cases and in 6 cases with Dacron patch. Postoperative complications were occurred in 8 cases [24.2%], and they were pleural effusion, congestive heart failure, and alopecia mainly. One case died due to air embolism postoperatively and operative mortality was 3.1%.

• Journal of Chest Surgery
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• v.11 no.3
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• pp.321-325
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• 1978

Coarctation of the aorta was rare condition among the congenital cardiovascular defects in Korea. We experienced a case of coarctatlon of the aorta [postductal type], which was successfully corrected by resection and end to end anastomosis. This patient, 21 years male patient, was admitted to the medical department for evaluation of hypertension, headache and exertional dyspnea during 4 years, and transferred to the department of chest surgery for operation. On physical examination, blood pressures were measured on both extremity, measuring 190/100mmHg on the arm and 100/80mmHg on the leg. Systolic murmur was heard on 2nd to 3rd left intercostal space and left sternal border. On simple chest x-ray, rib notching was seer/on low border of right 3rd and left 4th rib. Final preoperative diagnosis was made by the retrograde aortic catheterization and aortography, which showed the typical configuration of postductal type of coarctation with poststenotic dilatation of the aorta. On 20th, July, 1978, under the general anesthesia with endotracheal intubation, resection of coarctation of the aorta and end to end anastomosis was performed. During clamp for resection, blood pressure of upper extremity was elevated to 200/140mmHg, and controlled by Arfornad. During recovery, blood pressure over 160ramrig in systole was controlled by Reserpine for 8days postoperatively. At discharge, postoperative 8th day, brachial and femoral artery pressure was 145/85 mmHg and 135/80mmHg. After discharge, there was no evidence of specific symptoms and hypertension without antihypertensive drug.