• Journal of Chest Surgery
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• v.32 no.6
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• pp.504-509
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• 1999

Background: Heart transplantation is considerated for a selected certain group of complicated congenital heart disease in neonates because corrective surgery is very difficult and has high mortality. Precise planning of transplantation is necessary to adequately fit the donor heart to the recipient. Material and Method: We have performed 4 neonatal pig heart transplantations to test the technical feasibility. Experiment 1: The transplantation was performed using the same technique as the adult heart transplantation. Experiment 2: The transplantation for hypoplastic left heart syndrome was simulated as we reconstructed the whole aortic arch with donor aorta. Experiment 3: The heart transplantation was done with radical pulmonary artery reconstruction. Experiment 4: The experiment was performed for a long term survival. Result: Preoperative planning was very important for adequate fitting. All animals could be weaned from cardiopulmonary bypass, however, two animals died due to bleeding at pulmonary artery and left atrium. Conclusion: We concluded that the neonatal heart transplantation can be applied in some complicated Further using animal model is mandatory.

• Journal of Chest Surgery
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• v.34 no.3
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• pp.235-239
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• 2001

배경: 폐격리증, 선천성 낭성 유선종 기형, 기관지 낭종 선천성 엽기종 및 낭성 기관지 확장증 등의 폐에 발생하는 선청성 낭성 질환은 않으며 유사한 발생학적 및 임상적 양사을 가진다. 대상 및 방법: 1972년부터 1999년까지 본원 흉부외과에서 선청성 낭성 폐질환으로 수술받은 46명의 환자를 대상으로 병상 기록을 통한 임상상, 치료 및 병리소견을 검토하였다. 결과: 환자의 평균 연령은 16.2세 였고 남녀비는 17:29였다. 주 증상은 감염에 의한 발령이 11례, 호흡곤란이 10례 그리고 흉통과 가래 등이였고, 증상이 없었던 경우가 13례 였다. 질환은 폐격리증이 13례, 선청성 낭성 유선종 기형이 12례, 기관지성낭종이 12례, 낭성 기관지 확장증이 5례 그리고 선천성 엽기종이 4례였다. 수술은 단순 절계 8례, 폐구역 절제술 6례, 폐엽 절제술 31례 그리고 전폐 전제술 1례를 시행하였고 수술 사망은 폐엽 절제술은 시행한 7세된 기관지 낭종 환자로 수술직후 발생한 뇌경색으로 1례에서 발생하였으며 술후 합병증으로는 폐렴 4례와 지속적 공기누출과 농흉이 각 1례씩 이었다. 결론; 선청성 낭성 폐질환을 가진 환자들은 진단 즉시 수술하여 좋은 결과를 얻었다.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.361-363
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• 2009

A 17-year-old male patient was referred with symptoms of dyspnea. Multi-detector computerized tomography (MDCT) and echocardiography evaluation revealed quadricuspid aortic and pulmonary valves, an atrial septal defect (ASD), and pulmonary stenosis. We closed the ASD using a bovine patch and performed a commissurotomy of the pulmonary valve. Quadricuspid semilunar valves are very rare congenital abnormalities that are reported to occur nine times more frequently in the pulmonic valve than in the aortic valve. According to the Hurwitz Roberts classification, the aortic valve was type A, and the pulmonic valve was type B. The aortic valve normal function, but the pulmonic valve was stenotic and had abnormal function.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.56-59
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• 2002

The four most common types of congenital malformations involving the right atrium(RA) and the coronary sinus(CS) are congenital enlargement of the RA, single RA diverticulum, multiple diverticula of the RA, and aneurysm of the RA or CS. A previously healthy 6year-old child was presented with signs of upper respiratory tract infection. Chest X-ray and echocardiogram revealed a severely isolated right atrial enlargement. The abnormally dilated right atrim was widely resected under cardiopulmonary bypass. Pathology revealed multifocal myocardial loss associated with mild fibrotic changes of the endocardium and epicardium Our experience on this rare congenital disease is presented along with a review of the literature.

• Korean Journal of Clinical Laboratory Science
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• v.51 no.2
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• pp.265-269
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• 2019

Ebstein's anomaly is attached to the right ventricle with the tricuspid leaf attached to the lower right ventricle without any attachment to the tricuspid annulus. Most patients in their 20s are alive. Patients older than 25 years of age are similar in the incidence of heart failure and sudden death, and survive approximately 70% from 2 years, and 50% from 3 years, from 5% to over 50 years of age. Through this example, Ebstein's anomaly exists in various forms. A 22 year old case with WPW syndrome and those who survived to 77 years of age are reported. The normal heart structure in the apical four-chamber view should be observed on similar lines, even though the tricuspid annulus is slightly lower than the mitral annulus. If not, there will be some doubt regarding this anomaly. Furthermore, echocardiography plays an important role in the diagnosis and prognosis of congenital heart disease.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.514-517
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• 2003

Anomalous aortic origin of left coronary artery is a rare congenital coronary anomaly that can cause clinical manifestations such as ischemic chest pain, arrhythmic syncope or even sudden cardiac death. We describe a case of anomalous aortic origin of left main coronary artery presented as a cardiogenic shock which was successfully treated by coronary artery transfer.

• Neonatal Medicine
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• v.15 no.1
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• pp.89-93
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• 2008

Duplication of chromosome 12p has been rarely reported and are thought to be associated with congenital malformations and impaired development. We report a baby boy born with multiple dysmorphic features and congenital malformations. His karyotype was 46,XY, add(12)(p13.3). He has suffered from intrauterine growth restriction at birth. He showed abnormal cranio-facial findings such as microcephaly, hypognathia, clepft palate and low set ear. He presented with absence of uvula, micropenis and rocker bottom features of both feet, congenital heart disease, poor corticomedullary differentiation of kidney, and sensorineuronal hearing loss. We have been follow up him for seizure disorder and delayed development at out patient department.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.17-26
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• 1997

Objeitives. The surgical mortality of congenital heart defects has been reduced (or the very young age group. Especially, young age at repair is an important risk factor for mortality after repair of tetralogy of Falloff. Some risk factors were analyzed Methods. Three hundred and sixty six patients underwent surgical Intervention. Ages ranged from 5 days to 64 years, and 80 patients were adults(over 15 years of age). The defects consisted of 313(84.2%) acyanotic and 53(15.8%) cyanotic anomalies. The surgical mortalities were evaluated by univariate and multivariate analysis. Results. The overall surgical mortality was 10.4%. Mos deaths occurred in the infant group younger than 6 months(20/38 deaths) and in cyanotic group(21/38 deaths). Surgical infant mortality younger than 12 months was 24.8%(25/101). Risk factors of mortality in open heart surgery were age(p< 0.0001), body weight(p< 0.0001), pump time(p< 0.0001), aortic cross clamp time(p< 0.0001), use of total circulatory arrest(p<0.0001) and cyanotic disease(p<0.0001) by univariate analysis. But by multivariate analysis, the risk factor of mortality in open heart surgery was disease entity(p=0.002) only. A disease group with the highest risk was a cyanotic group(odds ratio was 15.3 relative to ventricular septal defect) excluding tetralogy of Falloff(odds ratio=0.27). Conclusions. Even though the most important risk factor was disease entity, we should conentrate our efforts on the technically improvable factors affecting surgical mortality indicated by univarlate analysis.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.509-512
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• 2009

Ebstein's anomaly is a complex congenital defect of the tricuspid valve and right ventricle. Various surgical methods to repair the regurgitant tricuspid valve have been reported, and most of them depend on monocuspidalization with using the anterior leaflet. We report here on our first experience with Ebstein's anomaly in a 31-year-old female patient who underwent cone reconstruction of the tricuspid valve with using three leaflets.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.554-560
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• 2003

Purpose : To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. Methods : 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. Results : The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. Conclusion : Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.