• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.633-636
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• 2005

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.175-182
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• 2006

VATER association is defined as a combination of 3 or more anomalies- vertebra (V), imperforate anus (A), esophageal atresia with or without tracheoesophageal fistula (TE), renal and radial anomaly(R). We reviewed our experiences in one center to determine etiology, prevalence, clinical manifestation, other associated anomaly and prognosis. Two hundred and twenty-three cases that underwent operations for imperforate anus or esophageal atresia were analyzed retrospectively through medical records at Department of Pediatric Surgery, Asan Medical Center from June, 1989 to July, 2005. The total number of neonates who had been admitted during period of study were 46,773 and VATER association was 9 (0.019 %, 1.92 persons per 10,000 neonates). Median gestational age and birth weight were $37^{+4}wk$ ($35^{+1}$ - $41^{+4}$) and 2,594 g (1,671-3,660), respectively and median age of mother was 32 years (23-38). There was no family history. Three patients were twins but their counterparts had no anomalies. Patients who have 3 anomalies were 6, 4 anomalies in two and 5 anomalies in one patient. Vertebra anomalies were detected in 7(77.7 %), imperforate anus in 8(88.9 %), esophageal atresia in 5 patients (55.6 %), renal anomaly in 6(66.7 %), and radial anomaly in 5(55.6 %), respectively. Four patients are alive, 2 patients were lost during follow up period. Three patients died due to neonatal sepsis, respiratory dysfunction and cardiac failure. VATER association did not appear to be a definite risk factor, but merely a randomized combination of 5 anomalies. The prognosis was dependent on the other associated anomalies, appropriateness of management and operation. Careful follow-up and aggressive treatmentare required for improving survival and quality of life.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.157-163
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• 1983

Ebstein's anomaly is a rare congenital cardiac malformation oand the ideal surgical correction seems controversial at present, and some problems are left unsolved in the surgical correction of this anomaly. Between June 1978 and June 1982, 12 patients with Ebsteins' anomaly underwent corrective open heart surgery at Seoul National University Hospital. Except for one patient, who had no ASD, all had a huge right atrium, secundum type ASD, and definite atrialized right ventricle. Typically, displaced tricuspid valve leaflets were found in all cases, but the degree of displacement and deformity were variable. In the point of NYHA functional classification, five were in class II, six were in class III, and one was in class IV. Ten patients were operated on by tricuspid valve replacement and pication. Two patients were operated on only by plication and annuloplasty techniques, and in all cases, ASD was closed. Postoperatively, four patients suffered from complete A-V block, and two of them died immediately. The remaining two patients took pacemaker generator implantation with good results. The other eight patients were in good condition. Tricuspid valve replacement using tissue valve and plication of the atrialized rght ventricle seems to be a good method of surgical correction for Ebstein's anomaly.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.895-897
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• 2006

Systemic arterial supply from the aorta to the lung is a rare congenital anomaly within the spectrum of bronchopulmonary sequestration according to Pryce's terminology. We describe our experience of this anomaly in an infant with congenital cardiac disease confirmed by multidetector CT scan. We found a systemic arterial supply from the aorta to the right lower lobe of lung without right lower lobar pulmonary artery and bronchopulmonary sequestration. This combination of congenital anomaly is most rare form.

• Journal of Chest Surgery
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• v.13 no.1
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• pp.1-12
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• 1980

In 1979 during the period of about 10 months 320 cases of open heart surgery were done in Seoul National University Hospital. There were 220 Congenital anomaly cases consisting of 113 acyanotic and 107 cyanotic varieties, and 1 O0 acquired cardiac lesions. Out of 100 acquired lesions 96 were valvular cues. Among 97 valve replacement cases 3 were Ebstein anomaly treated with plication and tricuspid valve replacement. Operative mortality rate for congenital anomaly was 10.6%, with 2.7% for acyanotic and 22.4% for cyanotic group. For acquired lesions over all operative mortality was 7%. Tetralogy of Fallot, ventricular septal defect, and atrial septal defect were the 3 main congenital anomalies, with 88 cues, 69 cases, and 27 cues respectively. In 61 simple ventricular septal defect without other anomalies operative mortality rate was 1.6%, in 27 atrial septal defect no death and, in tetralogy of Fallot 12.2%. Among 69 ventricular septal defect cases 19[27.5%] type I VSDs, after Kirklin-Becu classification, were found, rather high relative incidence of type I compared with Caucasian patients. Among 97 valve replacement cases 20 double valves were replaced-11 mitral with aortic and 9 mitral with tricuspid valves. Over all operative mortality rate for valve replacement was 8.2% with 3.3% in 61 mitral valve replace-merit. The over all operative mortality rate for 320 open heart surgery cases was 10.6%. Bubble type oxygenator and xenograft bioprosthetic valves were utilized In almost all cases.

• 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.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.288-292
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• 1985

The division of the right ventricle into two chambers by aberrant muscle bands traversing the sinus portion, Double Chambered Right Ventricle[DCRV], is a relatively rare congenital cardiac malformation. Double Outlet Right Ventricle[DORV], basically recognized by the origin of both great arteries from the morphologic right ventricle, is also a rare anomaly; its frequency has been reported as approximately 0.09 case per 1,000 birth. DORV associated with DCRV, unusual combination, is even more rare; only a few known cases have been recorded previously in the literature. This report presents our surgical experience with this rare anomaly, DORV with DCRV.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.448-454
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• 1980

The juxtaposition of atrial appendage may possibly be diagnosed by angiography recently, and it is widely regarded as an ominous sign of severe cyanotic congenital heart disease. However, it is apparently rare congenital cardiac anomaly in which the atrial appendages lie side by side, both to the left or right of the great arteries, known as left or right juxtaposition of the atrial appendages. Juxtaposition of the atrial appendages has no functional significance, since it does not, itself, cause any hemodynamic disturbance. But it`s presence always indicates the coexistence of other major cardiac anomalies. In review of literatures TGA and VSD are invariable present, and ASD is common. Other anomalies, such as, tricuspid atresia, pulmonary outflow that, obstruction bicuspid pulmonic valve, persistent SVC etc. are relatively high incidence. In this report, we present one case of 6 year old female child having left juxtaposition of atrial appendage combined with TGA [D-looping, D-transposition], TAPVD, large ASD, small VSD, and vertical vein.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.687-692
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• 1989

Aneurysm of the sinus of Valsalva is an uncommon cardiac anomaly, usually congenital in origin, which may occur as an isolated defect or in conjunction with other cardiac malformation. This report is a case of a ruptured sinus of Valsalva aneurysm with ventricular septal defect in a 18-year-old female patient who complained progressive exertional dyspnea. She underwent operative management using total cardiopulmonary bypass. The fistula originated from the right coronary sinus and ruptured into the right ventricle and coexistent lesion was supracristal ventricular septal defect. The repair was done through aortic and right ventricular approach. The ruptured sinus of Valsalva was closed with pledget suture and the ventricular septal defect was closed with patch. The postoperative result was good.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.162-168
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• 1990

Unroofed coronary sinus syndrome is an uncommon anomaly which is caused by incomplete formation of the left atriovenous fold and it is usually associated with persistent left superior vena cava. It may be diagnosed by cardiac catheterization and cineangiography but, if it is not diagnosed, it can bring out significant complications due to right to left shunt, such as brain abscess, cerebral embolism, transient ischemic attack, arterial desaturation and there will reduced patient`s life expectancy. Therefore corrective operation was needed. A case of unroofed coronary sinus syndrome which combines with valvular heart disease was experienced at the department of thoracic & cardiovascular surgery of Kosin medical college. The patient was 49 years old female and she complained dyspnea on exertion for 2 yrs. Cardiac catheterization with cineangiography and both superior venacavogram were performed for diagnosis and she was diagnosed as unroofed coronary sinus syndrome combined with mitral and tricuspid regurgitation. Surgical correction was accomplished by reroofing of coronary sinus with pericardial patch, closure of atrial septal defect and annuloplasty of both atrioventricular valves. Postoperative results were satisfactory and course of recovery was uneventful. We report a case of unroofed coronary sinus syndrome with review.