• Journal of Genetic Medicine
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• 제5권1호
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• pp.1-6
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• 2008

Knowledge of developmental biology is essential for clinicians who seek to develop a rational approach to the diagnostic evaluation of patients with birth defects. After an accurate diagnosis, a clinician can make predictions about prognosis, recommend management options, and provide an indication of recurrence risk for the parents and relatives. In this paper, we first review the basic mechanisms of embryological development and clinical dysmorphology. We then review cellular and molecular mechanisms in development and related congenital anomalies. Developmental anomalies have a major impact on public health. Genetic counseling and prenatal diagnosis, with the option to continue or to terminate a pregnancy, are important for helping families faced with the risk of a serious congenital anomaly in their offspring. Moreover, primary prevention of birth defects, for example, supplementation of prenatal folic acid and prevention of consumption of alcohol which has teratogenic effects, can be accomplished using developmental biology knowledge.

• Journal of Chest Surgery
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• 제13권1호
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• pp.34-40
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• 1980

Total 193 patients over 16 years of age who have underwent a surgical correction of congenital heart diseases during the period 1964 to September of 1979 were reviewed. 106 patients were male and 87 patients were female. 85 patients were in the ages of 16 years through 20 years. The oldest patient was 54 years old male who had atrial septal defect. The commonest defects were atrial septal defect that accounted for 66 cases [34.2%]. Ventricular septal defect was next one that accounted for 66 cases [34.2%]. Patients with tetralogy of Fallot defects were 34 cases [17.6%]. 25 cases had patent ductus arteriosus [13.0%]. Patients with pulmonary stenosis were 17 cases [8.8%] and transposition of the great arteries cases were 2 cases [1%]. There were 14 cases of operative death in this series. So operative mortality rate was 7.3%. The commonest cause of death was low output syndrome and next was renal failure. This reviewed series reveals the incidence of operable congenital heart defects appearing in adult cardiac surgical patients and an aggressive surgical approach can be justified with low operative mortality like as pediatric age group.

• Journal of Chest Surgery
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• 제21권4호
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• pp.757-760
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• 1988

Double mitral valve orifice is an unusual congenital abnormality, and is rarely of clinical importance. That abnormality not produce hemodynamic deficits, but it has been associated with other congenital heart diseases, especially atrioventricular canal defects, and so it is surgically important condition. After the first report by M.S. Greenfield in 1876, there are only a few reported cases all over the world. We experienced two cases of double mitral valve orifice with atrioventricular canal defect. The small accessory mitral orifice was placed in anterior mitral leaflet as a hole type in both cases. The A-V canal defects are corrected with a one patch technique, but the accessory orifices are left unclosed because there were no mitral regurgitations through the orifice. On the 4th postoperative day, the patients have been performed echocardiography; both had no mitral regurgitation. Both of patients are now being follow-up and we find no specific problems. A small accessory mitral orifice may be left unclosed and rarely produce significant regurgitation, conclusively.

• Journal of Chest Surgery
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• 제10권2호
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• pp.230-240
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• 1977

Atrial septal defect is one of the most frequently encountered congenital heart disease. Up to December 31, 1976, 1682 cardiac patients received cardiac catheterization in the cardiac department of Yonsei university medical college. Out of the 1682 cardiac patients 723 cases had congenital heart disease and only 116 cases had congetial atrial septal defect. This amounted to 16.04% of all those with congenital heart disease. 58 cases of congenital atrial septal defect operated in the chest surgery department were presented. Of these 58 cases of atrial septal defect, 27 cases were male and 31 cases were female. Their ages ranged from 5 years to 54 years. The systolic pressure of the main pulmonary artery of 40 out of the 58 cases of atrial septal defect was below 40% of that of the systemic blood pressure: in 6 cases, the range of the systolic pressure of the main pulmonary artery was 50-90mmHg; in 12 cases, the range of the systolic pressure of the main pulmonary artery was 40-50mmHg. Average age of these was 30. 1 years. This study tends to show that Korean patients with atrial septal defect even though younger have a slight higher systolic pressure of the main pulmonary artery than Western patients have. The pulmonary blood is 1.5-2.5 times of systemic blood flow in 52 cases out of 58 cases of atrial septal defect.In only one of the 58 cases of atrial septal defect, the Rp was found to be as high as 45% of Rs. All other cases were below this level.51 cases had ostium secundum defect, 4 out of these cases had ostium secundum defect combined with mitral incompetence and 6 out of them had double ostium secundum defect. The remaining 7 cases had ostium primum defect. Their atrial defects were repaired under direct vision utilizing extracorporeal circulation, by hemodilution technic combined with moderate hypothermia. 44 cases [2nd atrial septal defect] were repaired by direct sutures while 14 cases, including the 7 cases ostium primum defects needed patches [1 pericardium and 13 teflon patch]. In 4 cases there were single defects while showed two defects. However the associated septal defect was so small that it could be closed by direct sutures. The size of the defect ranged between 6.0cm2and 10.0cm2 in 19 cases[33.7%]: the smallest being 0. 5cm2 and the largest 24cm2. The surgical mortality was 2 cases [3.4%]. These one case with ostium primum defect, could not be resuscitated on operation table. The cause of death in this case was myocardial failure and MI. The other, a case of ostium primum defect had a second operation on the first operative day due to massive bleeding from LV vent-line insertion site.The patient died on 26th post-operative day due to sepsis.

• Neonatal Medicine
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• 제16권1호
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• pp.89-93
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• 2009

저자들은 당뇨병이 임신 초기부터 진단된 산모에서 출생한 신생아에서 작은 악안면, 우측소이증, 오른손의 손가락다지증, 오른쪽 눈물샘의 위치 이상이 동반된 상검판 기형, 근위부 공장폐쇄증을 지닌 사례와 수술 후 공장 부위의 협착증을 중재적 방사선 시술로 치료하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제53권1호
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• pp.16-21
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• 2020

Background: Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. Methods: The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. Results: Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. Conclusion: Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.

• Journal of Chest Surgery
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• 제11권1호
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• pp.46-57
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• 1978

Ventricular septal defect was the most common congenital Heart disease admitted to the Severance Hospital from December, 1963 to June 30. 1977. A total of 1,811 cardiac patients received cardiac catheterization in the CardiacCenter of Yonsei University Medical College. Out of the 1,811 cardiac patients, 791 cases had congenital heart disease and of these 172 cases had ventricular septal defect. This amounted to 21.7% of all those with congenital heart disease. Seventy cases of ventricular septal defect operated on in the chest surgery department are presented. Of these 70 cases of ventricular septal defect, 54 cases were male and 16 cases were female. Their ages ranged from 4/12 months to 32 years. The ratio of systolic pressure of the main pulmonary to systemic artery [Pp/Ps] for 29 of the 59 isolated ventricular septal defects was below 45 percent. Pp/Ps ratio for 19 of the 59 isolated ventricular septal defects was 75 percent. The patients were mostly below fifteen years of age. Out of 64 cases, there were 36 cases of type II defects, 20 cases of type I, 4 cases of type III , 2 cases of type IV, one case of both type II and one case of left ventricular right atrial communication. The anomalies associated with ventricular septal defect were 13 in all; 4 cases of aortic insufficiency, 3 cases of ruptured aneurysm of the sortie sinus of valsalva, 2 cases of ruptured aneurysm of the sortie sinus of valsalva with aortic insufficiency, 2 cases of patent ductus arteriosus, one case of ruptured aneurysm of the aortic sinus of valsalva with atrial septal defect [secundum type] and one case of atrial septal defect [secundum type]. Overall mortality was 8.6 percent. The mortality in pulmonary artery banding was 37.5 percent. The causes of mortality were in one case congestive heart failure, in one case asphyxia and in one case respiratory insufficiency. Tile mortality in ventricular septal defect associated with pulmonary hypertension was 7.1 percent. The cause of mortality was in one case low cardiac output syndrome. There was no mortality in the ventricular septal defects without pulmonary hypertension and mortality in the ventricular septal defect. In ventricular septal defect associated with combined anomalies, the causes mortality were in one case respiratory insufficiency and in one case congestive heart failure.

• 한국임상수의학회지
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• 제20권3호
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• pp.427-430
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• 2003

Akabane virus is a cause of severe congenital defects, but adult animals show no signs of infection. In this study, congenital abnormalitis associated with Akabane virus infection in Korean native goat. The prevalence of serum neutralizing antibodies to Akabane virus in goat population was investigated, indicating that approximately 30% of goats in Korea were seropositive(36/120). The mother goats have the highest titers of neutralizing antibodies, as 1:128. And also there showed seropositive of Akabane virus in newborn fetus fluids. The necropsy results of newborn fetus visceral organs were appeared normal. These findings provide that Akabane virus is the ethiological agent of congenital abnormalitis and stillbirth. Our results suggest that goat in natural situations are part of the Akabane virus transmission cycle.

• Child Health Nursing Research
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• 제25권3호
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• pp.262-272
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• 2019

Purpose: We aimed to identify factors influencing physical activity in adolescents with complex congenital heart disease. Methods: We recruited 92 adolescents with complex congenital heart disease from a tertiary medical center in Seoul, measured their levels of physical activity, and identified factors that influenced their physical activity levels using the Global Physical Activity Questionnaire, the New York Heart Association classification, congenital heart disease complexity, the Self-Efficacy Scale, and the Parental Bonding Instrument scale. Stepwise multiple linear regression was used to determine factors influencing physical activity. Results: Total physical activity was higher in males than in females (t=4.46, p<.001). Adolescents who participated in school physical education classes engaged in more physical activity than those who did not (t=6.77, p<.001). Higher self-efficacy (${\beta}=.41$, p<.001), male gender (${\beta}=.37$, p<.001) and participation in school physical education classes (${\beta}=.19$, p=.042) were associated with a higher likelihood of engagement in physical activity. Conclusion: It is necessary to develop nursing interventions that enhance self-efficacy in order to promote physical activity in adolescents with complex congenital heart disease. Physical activity should also be promoted in an individualized manner, taking into account gender, disease severity, and parental attitude.

• 동의생리병리학회지
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• 제21권2호
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• pp.561-566
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• 2007

After researching on infantile diseases in Hyungsang medicine, the writer got the conclusions as follows. The infants who are excess of the Yang energy need to nourish the Eum- blood. The main causes of the infantile disorders are congenital defect and malfunction of internal organs by nature, as results of these they suffer from mental disorders or being undergrown. And after birth they get ill from internal injury or external affections, mainly epilepsy by retention of undigested food, fever, cough, asthma, nasal obstruction, dermatopathia, and affection by cold, etc. In Hyungsang medicine Dam-body is apt to get ill from deficiency of Eum-blood and bangkwang-body from deficiency of Yang-energy. And infants are hare to be moderate in food, so they become to diseases of the Spleen and stomach, especially infants with Yangmyung type get to epilepsy, cough, skin disorders, and obese for the reasonof overeating. Among main infantile symptoms congenital defects, infantile mental disorders, and convulsive diseases come from congenital defect and malfunciton of internal organs, so it must be treated the symptoms following the reasons. Above all infantile mental disorders are treated not to separate the spirit from the body. And fever, cough and asthma, affection by cold, skin diseases, poor appetite, and obese come from deficiency of Kidney or the deficiency and excess of the Spleen and stomach. In order to prevent from infantile diseases right antenatal training, taking medicine rightly, exercise and eating good habits are needed to give guidance. Seeing through the clinical cases in Hyungsang medicine, we come to know that the infantile mental disorders come out primarily for the reasons of the congenital defect, and the infantile epilepsy come from malfunction of internal organs, and the nasal obstruction and skin diseases come from deficiency of Kidney or the deficiency and excess of the Spleen and stomach.