• Journal of Chest Surgery
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• v.49 no.1
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• pp.35-38
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• 2016

The management of recurrent tricuspid regurgitation after tricuspid valve repair in patients with Ebstein anomaly is difficult, and tricuspid valve replacement is most commonly performed in such patients. We report two cases of recurrent tricuspid regurgitation in patients with Ebstein anomaly that were successfully re-repaired using the cone technique. The cone repair technique is a useful surgical method for reconstructing a competent tricuspid valve, and can be applied in patients who have undergone previous tricuspid valve repair.

• Advances in pediatric surgery
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• v.16 no.2
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• pp.143-153
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• 2010

Congenital diaphragmatic disease is one of the common major congenital anomalies, and its mortality remained still high despite recent medical advances. The aim of this study is to examine the clinical characteristics of congenital diaphragmatic diseases. A total of 39 patients with congenital diaphragmatic disease that underwent surgery from January, 1997 to December, 2009 at Pusan National University Hospital were included in this study. Medical records were retrospectively reviewed. The male to female ratio was 30:9. Six out of 39 cases died (NS) before surgery, 17 patients had Bochdalek's hernia (BH), 11 patients hiatus hernia (HH), 4 diaphragmatic eventration (DE), and 1 Morgagni hernia (MH). There were no differences in mean birth weight and mean gestational age. NS (83.3 %). BH (35.3 %) was diagnosed more frequently than other diseases in the prenatal period. Three patients (17.6 %) of BH expired due to pulmonary hypoplasia and 1 patient had co-existing congenital heart disease. BH was diagnosed more frequently in the prenatal stage and had a higher motality rate than other conditions. Therefore, BH needs to be concentrated more than other anomalies.

• Journal of Chest Surgery
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• v.48 no.6
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• pp.407-410
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• 2015

A two-month-old infant presented with coarctation of the aorta, severe left ventricular dysfunction, and moderate to severe mitral regurgitation. Through median sternotomy, the aortic arch was repaired under cardiopulmonary bypass and regional cerebral perfusion. The patient was postoperatively supported with a left ventricular assist device for five days. Left ventricular function gradually improved, eventually recovering with the concomitant regression of mitral regurgitation. Prompt surgical repair of coarctation of the aorta is indicated for patients with severe left ventricular dysfunction. A central approach for surgical repair with a back-up left ventricular assist device is a safe and effective treatment strategy for these patients.

• Journal of Chest Surgery
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• v.24 no.8
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• pp.819-823
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• 1991

The congenital cystic adenomatoid malformation of the lung is a rare disease, and is one of the most common congenital lung diseases which require prompt surgical intervention. The prognosis depends on its tissue type, prompt diagnosis and surgical intervention. The lesion consists of enlarged, variable sized multiple cyst with overgrowth of terminal bronchioles, like hamartoma. This disease can be associated with other vascular anomalies or other congenital defect especially in type II lesion We recently experienced one case of congenital cystic adenomatoid malformation The patient was 2 months old infant who showed respiratory distress without associated anomaly. After right upper lobe lobectomy, the patient was recovered uneventfully.

• Journal of Chest Surgery
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• v.25 no.2
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• pp.167-175
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• 1992

During a 4 year period from 1987 to 1991, 152 patients underwent open heart surgery for congenital heart disease and acquired heart disease. Over all operative mortality was 6.6%. There were 76 congenital anomalies consisting of 73 acyanotic and 3 cyanotic and 76 acquired heart disease. The age distribution of 152 cases was 3 months to 62 years. Mean age was 13.0 years in congenital heart anomalies and 38.9 years in acquired heart disease patients.

• Journal of Chest Surgery
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• v.12 no.2
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• pp.89-92
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• 1979

Congenital multiple cystic disease of the lung is a loosely knitted clinical group of disease and shows various clinical and laboratory findings. It is hard to find out definite differences between the bronchogenic cyst and congenital multiple cystic disease of the lung in the embryologic developing process but we can accept the idea, the embryologic developing process is similar one. An 18 years old female patient had left lower lobe bronchiectasis and Rt. Mid. and lower lobe congenital multiple cystic disease of the lung. In BNUH chest surgery department, we managed this patient successfully by doing staged bilateral lung lobar resection.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.1-9
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• 2014

Many inborn errors of metabolism can be completely cured with early detection and early treatment. This is why neonatal screening on inborn errors of metabolism is implemented worldwide. In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia and congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU and congenital hypothyroidism to increase test numbers with same budget from 1995. 78 laboratories wanted to participate for neonatal screening test in 1999. Government decided to screen six items of PKU, congenital hypothyroidism, maple syrup urine disease, homocystinuria, galactosemia and congenital adrenal hyperplasia from 2006. In 2014, thirteen laboratories are participating. Inter laboratory quality control was started 6 times a year from 1994. In case a patient with an inherited metabolic disease is diagnosed by screening of government program, special milk is provided at government's expense. According to the government project, from 1997 to 2013, 7,080,569 newborns were screened. 144 PKU, 2.451 congenital hypothyroidism were detected. So incidence of PKU is 1/49,170 and congenital hypothyroidism is 1/2,888. The cost benefit of performing screening procedures coupled with treatment has been estimated to be as high as 1.77 times in PKU, 11.11 times in congenital hypothyroidism than cost without screening. By January 2007, many European countries had expanded of their newborn screening programs by inclusion of Tandem mass spectrometry. We are trying to increase the budget to test all newborns for Tandem mass spectrometry from 2016. We are considering four to five central laboratories which cover all newborns and are equipped with tandem mass spectrometer & enzyme immunoassay for TSH, 17OHP & enzyme colorimetric assay for galactose. And I hope to expand test including Wilson disease screening test and lysosomal storage diseases.

• Journal of Environmental Health Sciences
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• v.31 no.6
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• pp.451-456
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• 2005

Minamata disease made its first appearance in the world at Minamata City, Kumamoto Prefecture, in May 1956. In 1962 methyl mercury poisoning through the placenta was found for the first time in the world. This was called congenital Minamata disease. In all cases the clinical symptoms were consistent with those of cerebral palsy. The time and place of outbreak were the same as those for Minamata disease. Their mothers had eaten fish and shellfish during pregnancy. The principal symptoms of congenital Minamata disease are mentalretardation ($100\%$); primitive reflexes ($100\%$); disturbance of coordination ($100\%$); dysarthria ($100\%$); limb deformation (100%); growth disorders ($100\%$); nutritional disorders ($100\%$); chorea-athetose ($95\%$); and hypersalivation ($95\%$). However, today, when the world is polluted by mercury in various places and at various levels, the data we need is not represented by those severe cases, but rather by the chronic milder type. Even in Minamata, the issue of Minamata disease has not been resolved. And likewise, on a global scale the problem of Minamata disease is not yet over.

• Child Health Nursing Research
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• v.28 no.3
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• pp.187-196
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• 2022

Purpose: This study aimed to clarify the factors related to mothers' decisions to provide disease explanations to their children, investigate the associations between those factors and social adaptive skills, and examine support relative to children's growth and development. Methods: Data were collected from anonymous, self-administered questionnaires answered by 71 mothers of outpatient school-aged children with congenital heart disease. The questionnaire items included characteristics of mothers and children, decision factors for providing disease explanations, and children's social adaptive skills (Asahide-Shiki social adaptive skills test). Factor analysis was performed on the decision factors, and multiple regression analysis was performed to examine the association between the identified factors and each social adaptive skill. Results: The decision factors for providing disease explanations were the mother's explanation ability, the receptive capability of the child, and the child's interest and literacy regarding the explanation. Multiple regression analysis showed that language and social life skills were significantly associated with the child's receptive capability, and language and daily life skills were significantly associated with the child's interest and literacy regarding the explanation. Conclusion: Improving children's language, social life, and daily life skills may enhance their receptive capability and literacy regarding explanations of their disease.