• Journal of Chest Surgery
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• 제38권12호
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• pp.815-820
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• 2005

배경: 온전한 심실 중격을 가진 폐동맥 폐쇄증은 드문 선천성 심장 질환으로, 치료에 대한 다양한 접근 방법이 있어 왔지만, 아직까지 보편적으로 받아들여지는 방법은 없는 실정이다. 대상 및 방법: 1999년에서 2000년 사이에 온전한 심실 중격을 가진 폐동맥 폐쇄증을 진단 받고 수술한 14명의 신생아 환자를 대상으로 하였다. 수술 전 심초음파로 삼천판막의 크기를 측정하여 Z 값을 구하였다. 환자 모두에게 심초음파에 의한 추적관찰이 가능하였으며, 평균 추적관찰 기간은 46.0$\pm$9.5개월이었다. 결과: 14명의 환자들 중 2명의 조기 사망이 있었고, 1명의 환자는 1과 1/2심실교정의 시행 후 만기 사망하였다. 7명의 환자에서 양심실성 교정이 가능하였고, 이들의 Z값은 -0.8$\pm$1.50이었다. 1과 1/2심실 교정이 3명의 환자에게 시행되었으며, 이들의 Z값은 각각 -2, -2.5, -3이었다. Z값이 -4.5, -4.6인 2명의 환자에게 있어 단심실성 교정을 시행하였다. 이들 모두에 대한 심초음파 추적이 이루어졌으며, 남아 있는 심장 내의 결손으로 중등도의 폐동맥판막 폐쇄부전이 2명, 심한 정도의 폐동맥판막 폐쇄부전이 2명 있었다. 환자들의 우심실 기능은 비교적 괜찮았으며, 좌심실 기능은 정상이었다. 결론: 온전한 심실 중격을 가지고 있는 폐동맥 폐쇄증을 가지고 있는 신생아 환자들에게 있어 수술적 치료의 사망률은 그다지 높지 않았으며, 삼첨판막의 크기를 고려한 수술 방침의 결정은 비교적 안전하고 좋은 결과를 보일 수 있음을 알 수 있었다.

• Journal of Chest Surgery
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• 제21권5호
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• pp.816-827
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• 1988

Since 1978, We have experienced 87 cases of Fontan operations and the candidates of that increased in numbers recently with the improvement of the diagnostic and operative technique. We studied the prerequisite factors and hemodynamics of 22 cases of Fontan operations, done during the last one year period, which were 3 tricuspid atresia, 16 functional single ventricle and 3 anatomic single ventricle. The mean age was 68 months and the mortality rate 24%, and 9 patients of under 4 years of age were operated with 22.2% mortality rate, but the youngest, 16 months of age, patient survived well without problems. The preoperative pulmonary artery pressure[PAP], pulmonary vascular resistance[PVR] and postoperative right atrial pressure[RAP], left atrial pressure[LAP] value influenced the mortality, but age, preoperative Hb, preoperative PaO2 and pulmonary artery index[PAI] did not. There were favorable survival tendency in under 15mmHg of preop. PAP, 2a of preop. PVR and under 25cmHyO of postop. RAP, under 15cmHyO of postop LAP. The younger, the more pleural effusion and the longer postoperative admission days. The higher preop. Hb related to the higher postop. transpulmonary pressure gradient and the lower preop. PaO2 and PAI. The higher preop. PaO2, the less pleural effusion and postop. admission days. Preop. PAP closely related to preop. PVR and postop. LAP and high PVR increased the pleural effusion and postop. admission days. The larger PAI, the larger CI. We concluded that there were so many factors influencing the postoperative condition, but preop. PAP, PVR, Hb, postop. RAP and LAP were the most ones.

• Journal of Chest Surgery
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• 제22권4호
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• pp.594-600
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• 1989

Primary intracardiac repair of tetralogy of Fallot with low mortality and early good results, has been accomplished in recent years. But palliative procedures have been reserved for those hypoplastic pulmonary arteries, a hypoplastic left ventricle or anomalies of the coronary artery would make total correction difficult. And the Blalock-Taussig shunt operation is recognized as a standard and popular palliative procedure. I undertook a retrospective determination of the effect of the Blalock-Taussig shunt operation on the development of the main pulmonary artery and the right and left pulmonary arteries. Between January, 1980, and April, 1987, at the Severance Hospital, 16 patients were studied by cardiac catheterization and angiocardiography, before undergoing Blalock-Taussig shunting procedures for the palliation of severe symptoms of tetralogy of Fallot, and some time later, usually prior to a second procedure. The mean interval between catheterizations was 22.25 months. Patients with tetralogy of Fallot and pulmonary atresia or with an occluded shunt were not included. The primary and secondary angiograms of each patient were reviewed, and measurements of the diameter of the main pulmonary artery, the right and left pulmonary arteries, and the descending thoracic aorta were taken. The results are as follows; 1. The hematocrit decreased from 56.39% to 50.34%[p< 0.05], and the arterial oxygen saturation increased from 62.00 % to 81.31 %[p< 0.001] following shunt procedures 2. The ratio of the diameter of the right pulmonary artery plus the left pulmonary artery to the diameter of the descending thoracic aorta increased 1.30 k 0.28 times [p< 0.01]; but the ratio of the diameter of the main pulmonary artery to the diameter of the descending thoracic aorta increased 1.10 * 0.33 times, which was not. significant[p< 0.05]. 3. The interval between shunting and second catheterization was not related to the magnitude of change in the pulmonary arteries[r=0.141, p >0.05]. 4. The changes in the ratio of the diameter of the right pulmonary artery plus the diameter of the left pulmonary artery to the diameter of the descending thoracic aorta was inversely related to the initial ratio[r=0.757, p >0.001], but the change in the ratio of the diameter of the main pulmonary artery to the descending thoracic aorta was not related[r=0.059, p >0.05]. 5. There were no differences in enlargement of the pulmonary artery on the side of the shunt [ipsilateral] versus enlargement on the opposite side [p >0.05], nor according to the size of the shunt[p >0.05]. In conclusion, this study suggests that the Blalock-Taussig shunt is effective for the development of the right and left pulmonary arteries but not effective for the main pulmonary artery.

• Journal of Chest Surgery
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• 제35권4호
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• pp.303-306
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• 2002

심한 Ebstein기형이 있으면서 신생아시기에 증상이 있는 경우, 심한 심비대를 보이며 이로인해 폐발달의 장애가 생기고 기능적 폐동맥판폐쇄를 보이며, 동맥관의존 폐혈류를 보인다. 지금까지 이런 경우 양심실성교정은 실망적이었으며 근래에 와서 Fontan술식을 목표로 하는 Stames술식으로 좋은 결과를 보고하고 있다. 본원에서는 생후 4일째 심한 심비대와 함께 해부학적 폐동맥판폐쇄가 동반된 Ebstein기형의 환아에서 심방화된 우심실을 수직적 주름성형술, 삼첨판막륜성형술, 우심실유출로재건, 심방중격결손부분폐쇄, 우심방축소술로 양심실고정술을 시행하였다. 술후 환아는 심홉곽비의 현저한 감소와 심초음파검사상 경도의 삼첨판폐쇄부전을 보이며 10개월째 건강한 상태로 외래추적관찰중이다.

• Journal of Chest Surgery
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• 제33권4호
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• Journal of Chest Surgery
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• 제14권1호
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• pp.26-32
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• 1981

Three cases of tricuspid atresia were treated by Fontan varieties of operation in this department in 1980. The first case was 19 year old girl who underwent Glenn operation at the age of 6 years. Her second operation was done with ASD closure and 16mm Ionescu-Shiley valved conduit insertion between right atrium and main pulmonary artery. The second case was a 5 year old boy who underwent Kreutzer operation successfully utilizing 14mm Ionescu-Shiley va]ved conduit. The above mentioned 2 cases were Type Ib after Keith`s classification, whose immediate postoperative courses were complicated by pleura] effusion [in 2nd case chylothorax] hepatomegaly, and ascites. Those complications were relieved completely by medical treatment and closed thoractomy; Postoperative follow-up up to 11 months and 1 year periods were satisfactory with disappearance of cyanosis and dyspnea. The third case was a 8 year old boy who had complete TGA with TA [Keith`s Type IIb] who underwent Kreutze`s operation utilizing 14mm Ionescu-Shiley valved conduit, he died of low cardiac output Immediately after open heart surgery.

• Journal of Chest Surgery
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• 제16권4호
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• pp.431-443
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• 1983

A hundred and fifty-one patients with congenital heart disease less than 24 months old underwent intracardiac repairs from January 1982 to July 1983, which consists 24.2% of all the patients with congenital heart diseases operated during the same period. There were 98 patients[64.9%] with acyanotic congenital heart disease and 53 patients[35.1%] with cyanotic congenital heart disease, and 55 patients[36.4%] were less than 1 year of age. Twenty-two patients died within 30 days after surgery and 3 patients died after postoperative 30th day: Ventricular septal defect, four of 90 patients; Tetralogy of Fallot, five of 23 patients; Transposition of great arteries, nine of 17 patients; Tricuspid atresia, four of 5 patients; Pulmonary atresia, all of 2 patients; Single ventricle, one of single patients. Over all mortality was 16.6% and mortality of acyanotic congenital heart disease, cyanotic congenital heart disease and patients less then 1 year of age was 4.1%, 39.6% and 20.0% respectively. Still the mortality of cyanotic congenital heart disease is high. Careful preoperative evaluation of the detailed intracardiac anatomy and hemodynamics of the patients and proper selection of surgical treatment yield better clinical results.

• Journal of Chest Surgery
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• 제18권4호
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• pp.605-614
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• 1985

The principal aim of surgery for congenital heart anomalies is the establishment of normal hemodynamic function. Palliative and corrective operations are selected with time to attain this end with minimal risk. In recent years, as operative mortality after primary total correction is lower than the mortality after early palliation and delayed correction, corrective operations in infants have increasingly supplanted palliative ones. Two hundred and eighteen infants below 10 kg with congenital heart anomalies underwent primary surgical intervention at Yonsei Medical Center from March 1979 to June 1985. There were 155 infants with VSD, 35 Infants with TOF, 5 infants with ECD, 4 infants with TGV, 3 infants with DORV, 3 infants with Pulmonary atresia, 3 infants with ASD and PDA, 2 infants with DOLV, and the remainders were Sinus Valsalva rupture, residual mitral regurgitation after total correction of ECD, PAPVR, Cor triatriatum, Truncus arteriosus, and Tricuspid atresia. The overall surgical mortality was 15.1%. In the acyanotic group, 13 infants died among 168 infants, and mortality was 7.7%. But in the cyanotic group, the mortality rate was very high and 20 infants died among 50 infants raising the mortality to 40.0%. These poor surgical results in the cyanotic or complicated group was due to inaccurate diagnosis, improper surgical methods and inadequate post-operative care which should be improved.

• Journal of Chest Surgery
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• 제37권10호
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• pp.856-860
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• 2004

심내기형을 동반한 대동맥궁 단절은 매우 중한 자연경과를 갖고 있는 희귀한 선천성 심장 기형이다. 식도 폐쇄증과 기관 식도루를 동반한 심첨부 근육성 심실 중격 결손과 대동맥궁 단절을 생후 3일된 체중 2.6 kg의 신생아에서 단계적 수술법으로 치료한 경험을 보고한다. 1차 수술로서 우측 개흉술을 통한 식도 폐쇄증의 교정과 함께 좌측 개흉술에 의한 대동맥궁 광범위 단-단 문합술 및 폐동맥 교약술을 시행하였다. 1차 수술 후 87일째 정중 흉골 절개를 통해 심첨부 근육성 심실 중격 결손을 폐쇄하였다. 심실 중격 결손 폐쇄 전 유문부 근육절개술, 대동맥 전방고정술, 대동맥 풍선확장술 등의 추가 시술이 필요하였다. 최종 수술 후 3개월째 양호한 추적 결과를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제31권7호
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• pp.660-667
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• 1998

배경: 이 연구는 폐동맥 유출로 협착을 가진 다양한 복잡 심기형 환아에서 시행한 Lecompte 술식의 효과와 임상 적용에 관한 검증을 위한 것이다. 방법: 1988년 7월부터 1997년 12월까지 서울대학교 어린이 병원 흉부외과에서는 44명의 환아에 대하여 상기 술식을 시행하였다. 남녀비는 24대20이었으며 연령분포는 3개월에서 83개월까지로 평균 29.2개월이었다. 이 중, 심실 중격 결손과 폐동맥 협착(또는 폐쇄)을 동반한 대혈관 전위가 28명으로 가장 많았고(63.6%), 그 외 14명(31.8%)의 폐동맥 협착(또는 폐쇄)을 동반한 양 대혈관 우심실 기시 등이 있었다. 술식의 기본 원칙은 1)심실 중격 결손 부위의 확장 또는 누두부 중격 절제, 2)좌심실-대동맥 간의 심장내 도관 형성, 3)인공 심장외 도관을 사용하지 않는 폐동맥 간과 우심실의 직접 문합 등이었다. 결과: 대상 환자 중 3명에서 병원 내 사망이 있었으며 사인은 지속성 저산소증, 심근 부전, 패혈증으로 각각 판단되었다. 만기 사망 1명은 술 후 3개월에 패혈증에 의한 것이었다. 재수술은 6명에서 시행되었고, 폐동맥 유출로 협착 4명, 잔존한 근육성 심실 중격 결손 1명, 재발성 패혈성 식균증 1명 등이었다. Kaplan-Meier 법에 의한 누적 생존률은 1년, 2년, 4년 이후에 모두 92.7%였고, 재수술 없는 누적 생존률은 1년, 3년, 5년 이후에 각각 92.7%, 92.7%, 70.2%였다. 사망 위험 인자 분석 결과 대동맥 차단 시간이 통계적으로 의미가 있었고(p<0.05), 재발성 폐동맥 협착의 위험 인자인 연령, 폐동맥 지수, 유출로 재건에 사용된 재질 등은 모두 통계적으로 의미가 없었다(p>0.05). 결론: 이상의 연구에서 Lecompte 술식은 폐동맥 유출로 협착을 가진 다양한 복잡 심기형 환아에서 시행할 수 있는 효과적인 치료 술식이라는 결론을 얻었으며, 비교적 어린 나이에 완전 교정이 가능하고 사망률, 이환율 또한 수용 가능한 것으로 판단된다.