• Clinical and Experimental Pediatrics
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• 제49권8호
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• pp.895-897
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• 2006

폐 격리증이 없는 정상적인 폐에 체 혈관이 이상 기시하는 것은 매우 드문 질환이다. 저자들은 반복적인 하기도 감염을 보이는 영아에서 선천성 심장질환의 상태가 본 하기도 감염을 나타낼 정도로 심하지 않음을 이상히 여겨 컴퓨터 단층 촬영을 시행하였다. 그 결과 폐 격리증이 없는 폐의 이상 체 동맥 기시가 우측 폐하 엽에 존재함과 동시에 우측 하엽 폐동맥이 없는 사실을 확인하게 되었다. 어린 나이에 이 같은 선천성 질환의 조합은 이전에 보고 된 바가 없는 매우 드문 질환으로 생각된다.

• Journal of Chest Surgery
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• 제25권7호
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• pp.769-776
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• 1992

Total 632 cases of open heart surgery were performed in the department of thoracic and cardiovascular surgery, Pusan Paik Hospital, Inje University from october 1985 to december 1990. Among them, there were 503 cases of congenital heart disease and 129 cases of acquired heart disease. In the cases of 503 congenital heart disease surgically corrected, 122 cases over 15 years of age were reviewed and its results were summarized as follows ; l. In total 122 cases 65 were male and 52 female. 2. There were 54 patients under 20 years of age, 31 between 20 & 24 years, 20 between 25 & 29 and 17 over 30 years of age. The eldest was a 46-year-old female of atrial septal defect group. 3. There were 63 cases of VSD[51.7%], 38 of ASD[31.2%], 11 cases of TOF[9%] and so on. 4. In recovery cases, main post-operative complications were as follows; cardiac tamponade, arrhythmia, low cardiac output syndrome, pulmonary, edema pericarditis, mediastinitis, sepsis and DIC. 5. There were 3 cases of post-operative death in this series, so operative mortality rate was 2.5% compairing with 1.8% of pediatric cases below 15 years of age. 6. This reviewed series reveal that aggressive surgical approach in adult congenital heart disease can be justified with low operative mortality like as pediatric age group.

• 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.

• Journal of Chest Surgery
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• 제56권6호
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• pp.452-455
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• 2023

Patients who have undergone mechanical valve replacement require anticoagulation therapy with warfarin to prevent thromboembolism. However, administering warfarin to pregnant patients increases their risk of warfarin embryopathy or central nervous system disorders. Consequently, safer alternatives, such as heparin or low-molecular-weight heparin injection, are substituted for warfarin. However, limited research has been conducted on this subject, with no large-scale studies and particularly few investigations involving multiparous patients. A patient who had previously undergone mechanical mitral valve replacement for atrial septal defect and mitral stenosis received anticoagulant therapy with enoxaparin during 2 pregnancies. Upon confirmation of pregnancy, warfarin was replaced with subcutaneously injected enoxaparin with a dosage of 1 mg/kg at 12-hour intervals. The enoxaparin dosage was controlled using an anti-factor Xa assay, with a target range of 0.3-0.7 IU/mL. Intravenous heparin injections were administered starting 3 days prior to the expected delivery date and were continued until delivery, after which warfarin was resumed. No complications were observed during the deliveries.

• Journal of Chest Surgery
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• 제36권3호
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• pp.136-141
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• 2003

성인 선천성 심기형에 동반된 삼첨판막 폐쇄부전에 대한 판막륜 성형술의 중기 성적 및 잔존폐쇄부전의 위험인자를 분석하였다. 대상 및 방법： 1989년 8월부터 2001년 6월까지 총 73명의 성인환자가 선천성 심기형에 동반된 삼첨판막 폐쇄부전으로 판막륜 성형술을 받았다. 환자의 성비는 51：22로 여자가 많았고 연령은 16∼73세(평균 43세)였다. 진단은 심방중격결손(55), 심실중격결손(6), 부분 폐정맥 환류이상(4), 기타(8)의 순 이였다. 수술 전, 후 판막 폐쇄부전의 평균 혈류 속도는 각각 3.25 m/sec, 2.56 m/sec이었고, 판막 성형술의 방법은 De Vega 형(43), Kay 형(18), Ring Annuloplasty 형(12)의 순 이었다. 외래 추적기간은 2,347 patient-month (평균: 32.6개월)이었고, 이 기간 중 총 134예의 심초음파가 시행되었다. 초음파상 III/IV 이상의 잔존 판막 폐쇄 부전이 있는 경우 판막 성명술 실패로 간주하였다. 결과: 7명의 환자(9.6%)에서 판막 성명술 실패의 소견을 보였고, 이중 1명에서 삼천판막 치환술이 시행되었다. 잔존 폐쇄부전의 위험인자로는 심방 중격 결손 이외의 진단, 폐쇄부전이 우심실 압력 과부하에 의한 경우, 수술 후 높은 판막 폐쇄부전 혈류 속도 등으로 해석되었고. 판막 성형술의 방법은 잔존 폐쇄부전과 무관하였다. 결론: 선천성 심기형에 동반된 삼첨판막의 폐쇄부전이 우심실 압력 과부하에 의한 경우 판막 성형술 시 주의를 요하며, 수술 후 우심실 압부하가 소실되지 않는 경우 잔존 폐쇄부전의 위험이 높다.

• Journal of Chest Surgery
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• 제12권3호
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• pp.281-288
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• 제12권3호
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• pp.289-296
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• 제39권8호
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• pp.633-636
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• 2006

부분 심내막상 결손증은 일차공 결손과 승모판 전엽에 열공을 동반하는 질환으로 완전형 심내막상 결손증에 비해 임상 증상이 심하지 않으나, 승모판 부전이 진행할수록 심비대, 심부전, 폐동맥 고혈압 등이 더욱 쉽게 발현되어 그 예후가 좋지 않고, 따라서 고령에서는 보기 힘든 질환이다. 본원으로 30년 전부터 증상이 있던 67세 부분 심내막상 결손증 환자가 내원하였다. 심도자 검사상 폐동맥압이 45/22 mmHg, 평균 압력은 32 mmHg로 약간의 폐동맥 고혈압이 있었고, 수술 소견상 $3.5{\times}2.5\;cm$ 크기의 일차공 결손과 심한 석회화를 동반한 승모판 전엽의 열공이 보여 일차공 결손 첨포 봉합과 승모판막 치환술을 시행하였다. 수술 후 11일째부터 심한 방설 차단이 발생하여, 영구 심박동기를 삽입하였고, 수술 27일 후 퇴원하였다. 저자들은 임상적으로 보기 드문 고령에서의 부분 심내막상 결손증을 수술적으로 교정하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제55권3호
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• pp.206-213
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• 2022

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the Warden procedure, such as sinoatrial nodal dysfunction and systemic or pulmonary venous stenosis. We investigated the outcomes of the Warden procedure for repair of PAPVR to the SVC. Methods: This retrospective study included 22 consecutive patients who underwent the Warden procedure for PAPVR between 2002 and 2018. The median age and body weight at operation were 27.5 months (interquartile range [IQR], 5.0-56.8 months) and 13.2 kg (IQR, 6.5-16.0 kg), respectively. The median follow-up duration was 6.2 years (IQR, 3.5-11.6 years). Results: There were no cases of early or late mortality. No patients had postoperative heart rhythm problems, except 1 patient who showed transient sinoatrial nodal dysfunction in the immediate postoperative period. Procedure-related complications requiring reintervention occurred in 5 patients, including 3 of 4 SVC stenosis cases and 2 pulmonary venous stenosis cases during follow-up. The rate of freedom from reintervention related to the Warden procedure was 75.9% at 10 years. Conclusion: In cases requiring extension or creation of an atrial septal defect to achieve a sufficient venous pathway, or interposition of an entire circumferential conduit between the SVC and right atrium due to the shortness of the SVC in the Warden procedure, stenotic complications of the venous pathway occurred. Careful observation of changes in the pressure gradient or anatomical stenosis is required in such patients.

• Journal of Chest Surgery
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• 제36권12호
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• pp.911-920
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• 2003

본 연구에서 지난 17년간 경험한 부분방실중격결손증의 외과적 교정술의 장기결과를 후향적으로 분석하였다. 대상 및 방법: 1986년 4월부터 2002년 12월까지 부분방실중격결손증으로 외과적 교정술을 시행 받은 93명의 환자를 대상으로 사망률, 생존율 및 그 위험인자를 분석하고, 추적관찰 기간중 재수술과 관련되는 위험인자를 분석하였다. 남자 환자가 32명 여자 환자가 61명이었으며, 수술 당시 연령의 중앙값은 68개월(3∼818개월)이었고 평균추적관찰기간은 108$\pm$59.4개월(1∼200개월)이었다. 결과: 조기사망은 4예로 수술 사망률은 4.3%였다. 사망원인은 발작성 폐동맥고혈압증 1예, 저심박출증 1예, 심부전에 의한 심폐기이탈실패 1예, 심실세동 1예였고, 사망과 관련하여 통계적으로 의미 있는 인자는 없었다. 조기사망한 환자 4명을 제외한 89명의 환자를 추적 관찰하였고, 1예에서 만기사망을 확인하였다. 3년, 5년, 10년, 15년 생존율은 각각 95.7%, 94.3%, 94.3%, 94.3%였다. 수술 직후 63명(67.7%)의 환자에서 좌측 방실판막폐쇄부전이 개선되었고, 14명(15.1%)의 환자에서는 술 전과 같았고, 12명(12.9%)에서는 악화되었다. 추적관찰 기간 중 8명(9.0%)의 환자에서 재수술을 시행하였으며, 완전 교정술 후 평균 38.6개월(3∼136개월) 후 시행되었다. 3년, 5년, 10년, 15년 무재수술 생존율은 각각 94.0%, 91.4%, 91.4%, 88.2%였다. 재수술의 원인으로는 좌측 방실판막페쇄부전이 7예, 좌심실유출로협착이 2예, 잔존심방중격결손 1예, 좌측 방실판막협착 1예, 우심부전 1예였고, 재수술과 관련하여 좌심실 유출로 협착이 통계적으로 의미있는 위험인자였다(p=0.002). 10명의 환자에서 술 후 부정맥이 발생하였는데, 3명의 환자에서 상심실성 부정맥, 7명의 환자에서는 완전 방실전도차단이 발생하였고, 이 중 6명의 환자에서 영구 인공심박조율기의 삽입이 필요하였다. 걸론: 부분방실중격결손증은 낮은 사망률로 수술을 시행할 수 있었다. 재수술의 원인은 좌측 방실판막페쇄부전이 가장 많았다. 방실판막구조물의 기형이 동반된 경우 재수술의 가능성이 높아지는 경향을 보였으나 통계적 의미는 없었다. 또한, 술후 좌심실유출로협착여부가 재수술의 위험인자였으므로 좌측 방실판막 및 좌심실 유출로의 해부학적인 구조에 대한 정확한 이해가 요구된다. 좌심실유출로협착은 발생시 대동맥하부의 조직에 대한 절제술을 시행할 수 있으나 재발 가능성이 크고, 필요시 수정 Konno수술로 만족할만한 결과를 얻을 수 있었다. 또한, 방실전도차단은 초기시행단계에서 많이 발생하였으며, 심장 전도계에 대한 정확한 해부학적 이해와 경험축적으로 극복할 수 있었다.