• Journal of Chest Surgery
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• 제14권4호
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• pp.388-398
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

• Clinical and Experimental Pediatrics
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• 제46권2호
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• pp.154-161
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• 2003

목 적 : 수정 대혈관 전위 환자에서는 동반 심장 기형, 우심실 기능, 삼첨판 판막 기능, 부정맥 등이 예후에 영향을 주는 것으로 알려져 있다. 이 연구에서는 중기간의 관찰을 통해 여러 예후 인자들을 비교 분석하였다. 방 법 : 서울대학교병원에서 C-TGA로 진단 받은 94명을 대상으로 후향적 임상 기록 고찰을 시행하였다. 대상 환자들은 남아가 58명, 여아가 36명이었고, 추적 관찰 시 최종 평균 연령은 $12{\pm}9$세였으며, 평균 추적 관찰 기간은 $9{\pm}6.4$년이었다. 결 과 : 대상 환자 94명 중에 심장 수술은 72명이 시행 받았고, 고전적 교정술이 55례, 해부학적 교정술이 17례였다. 동반된 심장 기형(삼첨판 역류, 폐동맥 협착이나 폐쇄, 심실 중격 결손, 임상군), 심장 수술 여부와 종류, 완전 방실 전도 차단을 예후인자로 설정하였고, 예후 인자들 중에서 삼첨판 역류, 폐동맥 협착, 심실 중격 결손은 중등도 이상인 경우만 양성으로 채택하였다. 본 연구에서는 삼첨판 역류만이 생존율에 영향을 주는 예후인자였으며, Ebstein 기형과 고도 방실 전도 차단이 삼첨판 역류와 연관성이 있었다. 삼첨판 부전이 있는 환자의 20년 생존율은 35%로, 없는 환자 77%에 비교해서 유의한 차이를 보였고, 수술 직후 사망을 제외한 소그룹 분석에서도 삼첨판 부전이 있는 경우와 없는 경우의 20년 생존율이 각각 48%와 87%로 유의한 차이를 보였다. 20년 생존율 분석과 삼첨판 역류와의 연관성 분석에서 고전적 교정술과 해부학적 교정술간에 유의한 차이는 없었다. 결 론 : 삼첨판 부전은 수정 대혈관 전위 환자의 중요한 예후인자이며, Ebstein 기형과 방실 전도 장애가 삼첨판 부전에 영향을 줄 수 있다. 이 밖에 심장 수술, 동반 심기형 등 다른 예후인자에 대한 정확한 평가를 위해서는 장기적인 추적 관찰이 필요하다.

• Journal of Chest Surgery
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• 제38권4호
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• pp.284-290
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• 2005

본 연구에서는 엡스타인 기형에 대한 수술적 치료의 조기 및 중기 성적에 대하여 알아보고자 하였다. 대상 및 방법 1989년 1월부터 2004년 6월까지 총 29명의 환자들을 대상으로 하였다 중심 연령은 11.4세(4일$\~$50세)이었다. Grade 3 이상의 삼첨판막 폐쇄부전을 동반한 환자는 21명$(72.4\%)$이었다. 수술 방법은 삼첨판막 성형술 및 주름성형술 14예, Carpentier성형술 7예, 삼첨판막 치환술 4예, 체폐동맥 단락술 2예, 삼첨판막 성형술 1예, Fontan수술 1예이었으며, 양방향성 상대정맥-폐동맥 단락술이 5예에서 추가적으로 시행되었다. 신생아기에 수술을 받은 2명 중 1명은 양심실 교정을 받았고, 또 다른 1명은 체폐동맥 단락술을 시행 받았다. 조기 사망례를 제외한 28명의 환자들 중 21명에서 추적관찰이 가능하였으며$(75\%)$, 평균 추적기간은 37.6개월(3개월$\~$11.3년)이었다. 결론: 1명$(3.4\%)$의 조기 사망과 1명의 만기 사망이 발생하였다. 4명의 환자들에서 재수술이 시행되었다. 2명은 삼첨판막 재치환술을 시행 받았고, 2명은 삼첨판막 재성형술을 받았다. 추적관찰이 가능했던 21명의 환자중 grade 3 이상의 삼첨판막 폐쇄부전을 보인 환자는 2명이었으며, 대부분의 환자에서 현저한 증상의 개선을 보였다. 삼첨판막 치환술을 시행 받은 환자들을 제외하고 Kaplan-Meier 방법으로 산출한 삼첨판막과 관련한 재수술이 없을 확률은 1년 및 5년에 각각 $94.7\%,\;79.0\%$이었다. 결론: 대부분의 환자에서 판막 성형술이 가능하였으며, 이의 중기 성적은 비교적 양호하였으나 장기간의 추적관찰이 요구된다. 대부분의 환자에서 현저한 증상 및 혈역학적 개선을 보였다. 다섯 명의 환자들$(17\%)$에서 양방향성 상대정맥-폐동맥 단락술이 시행되었으며, 이의 적응증에 대한 추가적인 연구 및 추적 관찰이 필요하리라 생각한다.

• 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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• 제14권4호
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• pp.369-380
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• 1981

In this department 504 cases of valve replacement were done since 1968 to the end of October 1981. Since October 31, 1978 to the end of October 1981 ,333 Ionescu-Shiley bovine pericardial xenograft bioprosthetic cardiac valves were replaced in 265 patients. There were 149 males and 116 female. Ages ranged from 2 to 63 years with 25 cases under 15 years of age. Among 265 cases of Ionescu valve replacement there were 157 MVR, 36 VAR, 6 TVR, 45 MVR+ AVR, 16 MVR+TVR and 5 MVR+AVR+TVR cases with mortality of 5.7%, 8.3%, 16.7%, 8.9%, 18.8% and 20% for each group respectively. Over all mortality rate in 265 Ionescu valve replacement cases was 7.9% with 21 total deaths. Main causes of operative deaths were due to LCOS in 7, bleeding in 5, arrhythmia in 3, air embolism in 2,and heart block in 2 cases. There were 12 late complications with 6 deaths. Over all long-term survival rate was 89.8%. MVR showed the highest long-term survival rate with 92.4%, and MVR+AVR+TVR the lower with 80% lower with 80%.Average follow-up period was 14 month duration. Twenty five congenital anomaly cases were operated with Ionescu-valve replacement that consisted of 7 VSD+AI, and 5 Ebstein anomaly cases with over all operative mortality of 16% and late mortality of 14.3% among 21 operative survivors. There were 25 Ionescu valve replacement cases in pediatric patients under the age of 15 years, with 4 operative deaths. Fourteen MVR, 7 AVR, and 3 TVR cases were found. Even though long-term follow-up study was short in postoperative period with total of 33~.0 months among 244 operative survivors ranging one to 36 months, the late survivors showed beneficial long-term results two thromboembolic episodes in 244 patients were found. More cases and longer term follow-up study are warranted for valve replacement in pediatric and TVR cases with Ionescu-valves which have advantageous hemodynamic structures compared with other bio-prosthetic heart valves.

• Journal of Chest Surgery
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• 제34권8호
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• pp.597-603
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• 2001

본 논문을 쓰는 목적은 본원에서 2000년 9월까지 시행한 110례 심장수술을 대상으로 임상분석하여 향후 임상성적에 도움을 얻고자 하였다. 대상 및 방법: 본원에서는 1995년 1월부터 동맥관개존증수술을 시작한 이래 1999년 2월부터 개심술을 시행하여 2000년 9월까지 동맥관개존중 이중결찰술 10례, 개심술 100례 도합 110례의 심장수술을 시행하였다. 결과: 수술받은 환자들중 조선족이 74례(67.3%), 한족이 35례(31.8%)였다. 선천성심질환은 95례, 후천성심판막질환은 15례였다. 선천성심질화는 비청색성심질환이 83례로서 심실중격결손 및 연합기형 45례, 심방중격결손 및 연합기형 20례, 동맥관개존중 연합질환 11례, 동맥관개존증 연합질환 11례, 선천성대동맥판 협착 5례, 선천성 누두부협착 및 Ebstein 기형 각각 1례였다. 청색성심질환이 12례를 차지하였는데 11례가 활로씨사증후군으로서 전부 완전교정술을 시행하였다. 후천성심판막질환은 7례에서 판막치환술이 시행되였는데 그중 3례가 연합판막치환술, 3례가 승모판치환술, 1례가 대동맥판치환술이 진행되었다. 또한 8례에서 판막성형술을 시행했는데 승모판 성형술은 교련절개술, 판첨절제 성형술, 건삭성형, 유두근성형술과 판륜환을 이용한 판륜성형술 1례, 인공건삭 형성술 1례등이 포함되였다. 결론: 선천성심질환에서 비청색증군은 수술후 양호한 단기결과를 얻었으나 청색증군 활로씨사증후군은 수술방법에 따라 술후 회복의 차이가 다소 있었으며 향후 장기추적관찰을 요한다. 후천성심판막질환중 판막성형술의 단기결과는 비교적 양호하나 장기추적관찰을 요한다. 전체에서 수술사망은 한례도 없었다.

• Journal of Chest Surgery
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• 제28권5호
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• pp.447-452
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• 1995

From January 1983 to December 1992, 116 patients under 10Kg of body weight were underwent open heart surgery in National Medical Center.There were 64 males and 52 females, and their mean age was 14 2.6 months, ranged from 3 days to 38 months. Their mean body weight was 7.4$\pm$1.2Kg, ranged from 2.4Kg to 10Kg which were less than 3 percentile of normal developmental pattern in 109 out of 116 cases.The composition of the patients was VSD[73 , TOF[18 , ASD[8 , ECD[5 , TGA[4 , PS[3 , TAPVC[2 , TA[1 , cardiac rhabdomyoma with ASD[1 , and Ebstein`s anomaly[1 . The overall surgical mortality was 17.2%.In acynotic group, 11 patients died among 91 patients and the mortality was 12.1%, in cyanotic group 9 patients died among 25 patients, the mortality was 36%.The surgical result of patients under 5Kg of body weight was worse than those over 5Kg of body weight. [56.2% versus 11% The surgical result in this period was improved year by year, but poor surgical results in the group of cyanotic, under 5Kg of body weight should be improved by means of more precise preoperative diagnoses, more advanced surgical techniques and more proper postoperative management.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.554-560
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• 2003

목 적 : 계태의 발생 과정에서 감마선 조사에 의한 심장 기형유발 유무와 심장 기형의 종류를 알아보기 위하여 본 연구를 시행하였다. 방 법 : 토종 닭의 수정란을 사서 닦은 후 대조군에게는 감마선을 조사하지 않았다. 실험군으로 닭의 수정란을 인공 부화기에 넣고 3일 후(72시간 후)에 꺼내어 700 curie 1.25 Mev Cobalt 60에서 나오는 감마선을 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, 300 rad로 각각 60개의 수정란에 조사 후 인공 부화기에 넣어 총 3주간 지속시켰다. 온도 $38^{\circ}C$와 습도 55-70%로 유지하여 3주간 부화시킨 계태를 개흉하여 포르말린으로 고정하였고, 실물 현미경하에 해부하였다. 결 과 : 감마선 조사하지 않은 대조군에서 생존율은 76.4%이었고, 심장 기형은 발견되지 않았다. 실험군에서는 50 rad에서 300 rad로 선량이 강할수록 생존율은 63.3%에서 33.3%로 유의하게 감소하였고(P<0.001), 심장 기형의 발생률도 생존 계태의 7.8%에서 25.0%로 유의하게 증가하였다(P<0.001). 선량이 증가 할수록 심실중격결손의 크기가 증가하고 복잡기형이 나타나는 경향을 보였다(P<0.001). 대혈관 기형도 이상기시, 저형성, 형성부전으로 심해지는 경향을 보였다. 결 론 : 계태 발생 과정 중 감마선 조사의 시기와 선량이 선천성 심장병의 발생에 중요한 영향을 미친다. 조사 시기를 3일 후로 고정한 후 선량이 증가함에 따라 생존율은 감소하였으며, 선천성 심장병의 빈도는 증가하였고, 복잡 심장 기형이 생겼다. 또한 감마선 조사에 의해서 계태에서 혈관 기형을 유발할 수 있었다. 감마선 조사는 계태에 직접적인 조작을 하지 않고 또한 혈역학적 변화를 직접 유도하지 않는 실험이므로 거대분자인 핵산의 변화에 의해서 심장 기형과 대혈관 기형이 발생하였을 것으로 생각하며 기형 형성의 기전을 밝히기 위해 앞으로 염색체와 분자유전학적 연구가 필요할 것이다.

• Journal of Chest Surgery
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• 제26권4호
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• pp.282-293
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• 1993

From Sep. 1985 to Dec. 1992, total 1000 cases of open heart surgery [OHS] were performed in the department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College Of Medicine, Inje University.Among the total 1,000 cases of OHS, there were 823 cases with congenital heart diseases [CHD] and 177 cases with acquired heart diseases [AHD]. The age distribution was 9 days [4.0 kg] to 49 years in CHD and 11 to 64 years in AHD, In the 823 cases of CHD, there were 763 acyanotic cases and 60 cyanotic cases. The CHD cases consisted of 520 VSD [63.2 %], 177 ASD [21.5 %], 60 TOF[7.3 %], 27 PS [3.3 %], 17 ECD [2.1%], 7 Valsalva sinus rupture [0.9 %], 4 TGA [0.5 %], 3 Ebstein`s anomaly [0.4%], 3 DORV[0.4%], and others. The corrective operations were applied for congenital heart disease with the result of 2. 8 % hospital mortality. In the 177 AHD, 168 cases were valvular heart diseases, 7 cases were cardiac tumors and one LA thrombus and one annuloaortic ectasia. In the 168 valvular heart diseases, there were 115 single valve replacement cases [16 AVR, 99 MVR], 20 cases of double valve replacement[AVR & MVR], 15 cases of MVR with TVA, and 10 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 199`. In MVR, 66 of St. Jude Medical valves, 78 ofCarpentier-Edward valves, and 5 of Ionescu-Shiley valves were used. In AVR, 38 of St. Jude Medical valves and 12 of Carpentier-Edward valves were used.The hospital operation mortality rate of congenital acyanotic, cyanotic, and acquired heart diseases were 1.6%, 18.3 % and 3.4% respectively. The overall mortality rate was 2.9 % [29/1000].

• Journal of Chest Surgery
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• 제13권4호
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• pp.422-434
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• 1980

Since 1968 up to the end of October 1980, 448 valves were replaced in 354 patients in Seoul National University Hospital. There were 238 mitral, 38 aortic, 7 tricuspid, 45 aortic with mitral, 23 tricuspid with mitral, and 3 triple valve replacement aortic mitral and tricuspid cases. Annual increase of mitral valve replacement cases and decrease of operative maortality were remarkable. Recently operative mortality of mitral valve replacement is about 5%. Sex ratio of mitral valve replacement is almost equal and there were 12 cases of pediatric patients (5%) among 238 cases, and patients under the age of 20 years were 34 (14.3%). Mitral valve replacement was done for 199 single mitral, 38 double valve and one triple valve lesions. Among 238 mitral valve replacement paients left atrial thrombus in 23(9.7%), atrial fibrillation in 132 (55.5%), and reoperation after blind mitral commissurotomy in 12(5%) cases were noted. In recent cases bioprosthetic valves, mainly lonescu-shiley valve were utilized to overcome the difficulties of postoperative late complications in anticoagnuation, especially for the rural patients and pediatric cases, in addition to the hemodynamic advantages of lonesocu valve. Among 354 patients 16 cases were congenital heart anomaly related, 5 ventricular septal defect related aortic and 4 Ebstein related tribuspid valve replacement cases. There were 2 congenital anomaly related mitral valve replacements, one for congenital mitral insufficiency of 7 years old boy and one for corrected transposition of the great vessels associated with mitral insufficiency. Among total 354 valve replacements 49 operative deaths (13.3%) were noted and in 238 mitral valve replacement 24 operative deaths occurred (10.1%). In 39 patients among 354 total valve replacements late complications were found. In 238 mitral valve replacement cases late complications were noted in 26 patients, among whom 16 cases expired. Main late complications were thrombe-embolism, subacute becteerial endocarditis, arrythmia cerebral hemorrhage due to unsatisfactory anticoagulation, and congestive heart failure in the incipient period of valve replacement were also noted. In mitral valve replacement cases long-term survival rate was 83.2% who showed marked clinical improvement. Ther were no evidences of calcification during the 2 years follow-up period for the lonescu-valve replacement cases among 19 pediatric patients. In conclusion 238 cases of mitral valve replacement were done with 24 operative deaths and 26 late complication cases among whom 16 expired. The long term survival was 83.2% of the cases. In pediatric cases in place of coumadin anticoagulation Persantin **** 75 and aspirin were administered after valve replacement. In adult cases who have difficulaties with coumadin anticoagulation and for those even with bioprosthetic heart valve replacement who needs long-term or permanent anticoagulation persantin 75 and aspirin combination regimen were administered with antisfactory results.