• 제목/요약/키워드: Congenital defect

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정상적인 해부학적 연결을 가진 선천성 폐정맥 협착증 -치험 1예 보고- (Congenital Pulmonary Vein Stenosis with Normal Anatomical Connection -One case report-)

  • 박준석;장윤희;정미진;강이석;전태국
    • Journal of Chest Surgery
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    • 제37권4호
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    • pp.364-368
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    • 2004
  • 선천성 폐정맥 협착증은 매우 드문 기형으로 진행성 폐동맥 고혈압 및 조기 심부전으로 사망률이 높아 적극적인 내외과적 치료가 요구된다 수술적 치료법으로는 품선 확장술, 스텐트 삽입술, 전폐절제술, 폐이식술, 패취 봉합술, 그리고 무봉합 교정술 등이 있다. 본원에서는 정상적인 해부학적 연결을 가진 폐정맥 협착증에서 무봉합 교정술 및 수술 후 Sildenafil, Iloprost 그리고 NO gas등의 폐혈관 확장제를 사용하여 성공적으로 치료한 1예를 경험하여 이를 보고하고자 한다.

심폐기를 이용한 심방중격결손의 외과적 교정: 1례 보고 (Surgical Repair of Atrial Septal Defect Using Extracorporeal Circulation: One Case Report)

  • 이동준
    • Journal of Chest Surgery
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    • 제10권1호
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    • pp.143-147
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    • 1977
  • Atrial septal defect is the most common of the congenital heart disease in the adult. Since the first description of atrial septal defect by Rokitansky in 1857, this anomaly has been studied by many workers in past one century. In 1953, Lewis had first corrected the atrial septal defect under direct vision with deep hypothermia, and Gibbon [1954] had first done the atrial septal defect under direct vision with extracorporeal circulation. In our college [May 2’ 1977], we have first repaired the A.S.D. under direct vision with artificial heart-lung machine and, the defect was 4x5cm in size which was closed by Dacron patch. This patient was 12 year old girl who is well now. [postoperative 13 days]

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방실중격결손증의 외과적 치료 (Surgical Treatment of Atrioventricular Septal Defect)

  • 이광숙
    • Journal of Chest Surgery
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    • 제22권6호
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    • pp.990-995
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    • 1989
  • Since 1984, 24 patients underwent repair of atrioventricular septal defect. Nineteen had a partial defect and 5 had a complete atrioventricular septal defect. There were 9 men and 15 women, ranging in age from 1 to 50 years [mean age, 13.3 years]. Four patients had a Downs syndrome. Additional congenital heart defects were present in 11 patients. One patient had palliative operation prior to total correction. In partial defects, the primum atrial septal defect was closed with Xenomedica patch and the mitral valve was repaired with simple closure of the septal commissure. Central incompetence from annular dilatation was repaired by a local annuloplasty. In complete defect, the septal defects were closed with two patches except one. Operative mortality was 5% in partial defects and 60% in complete defects and low cardiac output was the commonest etiology. In a mean follow-up period of 27.9 months [range, 4 to 63 months] there were no late death and no instances of late-onset complete heart block. One patient required reoperation [MVR] for residual mitral regurgitation. The majority of patients were asymptomatic and mean postop. NYHA functional class was 1.2.

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개심술 치험 보고: 12예 (Clinical experience of open heart surgery: 12 cases)

  • 최영호
    • Journal of Chest Surgery
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    • 제16권3호
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    • pp.289-294
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    • 1983
  • The report is concerned to our experience of 12 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Chosun University Hospital during the period between Nov, 1979 and April, 1983. 1. There were 4 cases of congenital anomaly and 8 cases of acquired heart disease. 2. There were 6 male and 6 female patients with a mean age of 20 years. [range 9 to 33 years]. 3. The cases induced 2 ventricular septal defect, 2 atrial septal defects and 8 acquired valvular heart diseases. 4. The surgical managements were 2 primary repair for atrial septal defect and 2 patch closure for ventricular septal defect, 1 triple valve replacement [AVR MVR TVR], 1 aortic valve replacement, 4 double valve replacement [AVR MVR] and 2 open mitral commissurotomy for pure mitral stenosis. 5. The average cardiopulmonary bypass time was 61.5 minutes for congenital heart disease and 201.4 minutes for acquired valvular heart disease and the average aortic cross clamping time was 36.75 minutes for the former and 165.6 minutes for the latter. 6. Postoperatively, there were 1 Alopecia, 1 Electric burn and 1 wound infection as complication. 7. Overall operative mortality was 8.3%. 7. All patients received valve replacement were recommended anticoagulation with persantin.

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성인에서 선천성 심장기형의 수술성적 -725 치험례 보고- (Operative result congenital cardiac disease in adult - A reort of 725 surgical cases-)

  • 이정렬;서경필
    • Journal of Chest Surgery
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    • 제19권1호
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    • pp.116-121
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    • 1986
  • Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

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피부계 이상을 동반하는 선천성대사질환 (Congenital Metabolic Disorders with Cutaneous Changes)

  • 이상은
    • 대한유전성대사질환학회지
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    • 제22권2호
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    • pp.53-57
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    • 2022
  • 다양한 아미노산 및 지질 대사 질환에서 피부와 모발의 변화가 관찰된다. 탈모증이 관찰되는 경우 장병성 선단피부염(아연 대사 장애), 비오티니다아제 결핍증 (비타민 B), 다발성 카르복실라제 결핍증, acrodermatitis acidemica 등 아미노산 및 비타민 대사 결함을 의심해볼 수 있다. 또한 부서지기 쉬운 모발이 관찰되는 경우 아르기니노숙신산뇨증 또는 시트룰린혈증 및 점액다당증을 의심해볼 수 있다. 건조하고 두꺼워진 인설을 가진 피부 또는 비늘증은 중성지질 축적 질환, 지방산 대사 장애, 콜레스테롤 합성 및 대사 장애와 관련하여 나타날 수 있다. 수포성 병변은 장병성 선단피부염, 비오티니다아제 결핍증, 홀로카르복실라제 합성효소 결핍증, acrodermatitis acidemica 등에서 나타날 수 있다.

심장판막증이 동반된 관상동정맥루 -수술치험 1례- (Coronary Arteriovenous Fistula Associated with Valvular Heart Disease)

  • 임승현
    • Journal of Chest Surgery
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    • 제27권7호
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    • pp.624-627
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    • 1994
  • Congenital coronary arteriovenous fistula is a rare cardiac defect that causes coronary arterial flow to drain into the right cardiac chambers, the pulmonary artery, the coronary sinus, or the left cardiac chambers. The most frequently involved vessel is the right coronary artery. We experienced a case that had a coronary arteriovenous fistula associated with valvular heart disease. With the cardiopulmonary bypass done under hypothermia, mitral valve replacement was accomplished and the fistulas of both proximal and distal portions of the right coronary artery were closed with 3-0 prolene. Postoperative course was uneventful.

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선천성 횡격막탈장 :수술 치험2례 (Congenital postrolateral diaphragmatic hernia; a report of two cases)

  • 전찬규
    • Journal of Chest Surgery
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    • 제28권6호
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    • pp.640-643
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    • 1995
  • Bochdalek hernia is the congenital posterolateral defect in the diaphragm caused by a failure of the pleuroperitoneal canal to close at 8 weeks, gestation. Infants with hernia diagnosed at birth have poor prognosis. Survival rate depends on pulmonary growth and development, preoperative stability and postoperative care. We experienced two cases of Bochdalek hernia, one in right was repaired with patch closure using sheet and the other in left was repaired with simple closure.

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심혈관 질환 214예의 수술치험에 관한 임상적 고찰 (Clinical analysis of 214 cardiovascular surgeries)

  • 이철주
    • Journal of Chest Surgery
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    • 제19권4호
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    • pp.672-677
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    • 1986
  • From April, 1984 to August, 1986, 214 cases of cardiovascular surgeries had been performed at Yeungnam University Hospital consisting 158 open heart surgeries and 56 non-open heart surgeries. The leading cardiac anomaly of open heart surgeries was ventricular septal defect which was 43% of congenital heart diseases, and most of remaining non-open heart surgeries were ligating patent ductus arteriosus. We had observed 33 postoperative complications such as wound problems, transient arrhythmia, postpericardiotomy syndrome, bleeding requiring reoperation and so on. 3 cases of surgical mortality were present [2 in congenital heart diseases and 1 in acquired heart disease], which resulting 1.9% of surgical mortality rate in the open heart surgeries.

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