• Journal of Chest Surgery
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• 제29권2호
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• pp.171-176
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• 1996

1983년 3월부터 1994년 6월까지 22명의 환자에게 관상동맥 우회로 조성수술과 병행한 개심수술을 시행하였다. 대상환자들의 연령은 42세에서 72세 사이로 평균 60.4$\pm$8.2세였다. 이중 17명의 환자가 남자였고, 여자환자는 5명이 었다. 좌심실 박출계수는 25%에서 65% 사이로 평균 46.9 $\pm$ 14.2%였다. 22명의 대상환자중 9명이 심근경색증의 합병증으로 발생한 기계적인 장애를 동반하고 있었으며, 이중 5명은 좌심실류, 3명은 심실중격 결손 그리고 1명은 유두근 파열에 의한승모판 폐쇄부전증이 롱반되었다. 나머지 대상환자중 9명은 류마티스성 판막질환(대동맥판막 질환 7명, 승모판막 질환 2명)을 동반하였고, 2명은 좌심방혈전, 1명은 심방중격 결손 그리고 1명은 상행대동맥류를 동반하였다. 관상동맥 이식 편수는 1개에서 4개 사이로 평균 2.1$\pm$1.0개였다 수술후 합병증은 3명에서 발생하였으며, 이중 2명은 수술전후 심근경색증, 1명은 하지의 창상감염이었다. 수술사망은 1명에서 발생하였으며, 사망원인은 수술전후 심근경색증에 의한 저심박출량에 기인하였다. 추적조사 기간은 3개월에서 136개월 사이로 평균 41.1$\pm$ 40.2개월이었으며, 이기간 동안 1명\ulcorner 사망하였다. 사망원인은 뇌출혈이었다. 장기생존한 20명의 New York Heart Association functional class는 모두 I과 II였다. 비록 대상환자의 수가 적다고는 하지만 저자들의 수술결과는 양호한 것으로, 따라서 저자들은 관상동맥 우회로 조성수술을 병행한 개심수술이 수술위험도가 높지 않으면서 만기 사망율도 줄일 수 있을 것으로 생각한다.

• Journal of Chest Surgery
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• 제13권2호
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• pp.105-109
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• 1980

Tetralogy of Fallot has shown its familial aggregation in several familial studies. This reported case is another example which occurred in a family in two brothers. They revealed no cytogenetic abnormalities, but the anatomical cardiac malformation of them showed much similarity, total conal defect in ventricular septum and .one had patent foramen ovale, the other atrial septal defect. The familial recurrence tendency of Tetralogy of Fallot as well as other congenital heart diseases could be explained on multifactorial inheritance as shown in many reports. In spite that we couldn`t find out any environmental trigger or teratogens, our case may be accepted on the base of multifactorial mechanism.

• Journal of Chest Surgery
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• 제20권2호
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• pp.323-327
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• 1987

Majority of patients with total anomalous pulmonary venous drainage [TAPVD] have severe symptoms within the first few months of life. And early in the experience with correction of TAPVD, hospital mortality was high, especially in infant. In Sejong General Hospital, we operated on 3 infants with TAPVD of cardiac type. Repair was performed under the conventional cardiopulmonary by pass in one case and by the total circulatory arrest in other 2 cases. Interatial septum between enlarged coronary sinus opening and interatrial septal defect was excised and the coronary sinus was radically unroofed to make wide opening between left atrium and common pulmonary venous channel. The defect in atrial septum was closed with redundant pericardial patch. Postoperative courses were uneventful except transient dysrrhythmia of A-V dissociation. They are doing well on follow up check.

• Journal of Chest Surgery
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• 제50권2호
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• pp.78-85
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• 2017

Background: Closure of a secundum atrial septal defect (ASD) is possible through surgical intervention or device placement. During surgical intervention, concomitant pathologies are corrected. The present study was conducted to investigate the outcomes of surgical ASD closure, to determine the risk factors of mortality, and establish the effects of concomitant disease correction. Methods: Between October 1989 and October 2009, 693 adults underwent surgery for secundum ASD. Their mean age was $40.9{\pm}13.1years$, and 199 (28.7%) were male. Preoperatively, atrial fibrillation was noted in 39 patients (5.6%) and significant tricuspid regurgitation (TR) in 137 patients (19.8%). The mean follow-up duration was $12.4{\pm}4.7years$. Results: There was no 30-day mortality. The 1-, 5-, 10-, and 20-year survival rates were 99.4%, 96.8%, 94.5%, and 81.6%, respectively. In multivariate analysis, significant preoperative TR (hazard ratio [HR], 1.95; 95% confidence interval [CI], 1.09 to 3.16; p=0.023) and preoperative age (HR, 1.04; 95% CI, 1.01 to 1.06; p=0.001) were independent risk factors for late mortality. The TR grade significantly decreased after ASD closure with tricuspid repair. However, in patients with more than mild TR, repair was not associated with improved long-term survival (p=0.518). Conclusion: Surgical ASD closure is safe. Significant preoperative TR and age showed a strong negative correlation with survival. Our data showed that tricuspid valve repair improved the TR grade effectively. However, no effect on long-term survival was found. Therefore, early surgery before the development of significant TR mat be beneficial for improving postoperative survival.

• Journal of Chest Surgery
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• 제25권1호
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• pp.32-41
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• 1992

From August 1982 to December 1991, 58 consecutive infants with tetralogy of Fallot underwent primary repair. Age ranged from 22 days to twelve months [n=58, 8.7$\pm$2.7 months] and body weight from 3.1 to 13 kilograms [n=58, 7.8$\pm$1.7 kilograms]. Qne infant had absence of the pulmonary valve; one had Ebstein`s anomaly and one had supramitral ring. Thirty-two patients [56%] experienced anoxic spell. Preoperative pulmonary artery indices were measured in 38 cases, ranging 126-552mm2/M2BSA[n=38, 251$\pm$79mm2/M2BSA]. All infants required a right ventricular outflow tract patch; in 41, the patch extended across the pulmonary valve annulus, in 13 of them, monocusps were constructed. All had patch closure of ventricular septal defect. Two infants had REV operation for avoiding injury to the canal branch of the right coronary artery which cross the right ventricular out flow tract. Post repair PRV/LV were measured at operating room in 40 cases, which revealed mean value of 0.49$\pm$0.12 [range: 0.25-0.74]. The hospital mortality was 10.3% [6 patients], and causes of deaths were right heart failure due to sustained right ventricular hypertension[4] and right ventricular outflow tract obstruction, intractablesuraventricular tachyarrhythmia[1], hypoxia[1] due to residual right to left shunt across the atrial septal defect in patient associated with Ebstein`s anomaly. All infants were doing well at follow-up from 1 to 101 months[20.6 months /patient, 1, 072 patient-month] Serial postoperative echocardiograms revealed no residual ventricular septal defects and estimated RVOT gradients between 0 and 40 mmHg except 3 cases [50, 50, 60 mmHg]. There were no late deaths and late ventricular arrhythmias or congestive heart failure. Redo operations were done in 2 cases because of residual right ventricular outflow tract obstruction. This experience with infants with tetralogy of Fallot suggests that, if mortality is tolerable, eletive repair of tetralogy of Fallot could be reasonably undertaken during the first year of life, and even better results could be anticipated along with improvement of methods of myocardial protection and postoperative care.

• Journal of Chest Surgery
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• 제31권2호
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• pp.168-172
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• 1998

하정맥동 결손(inferior sinus venosus defect)은 하대정맥과 우심방의 경계 부위에 발생하는 심방중격결손의 한 형태로 매우 드문 선천성 심장질환이며, 심방중격의 하부 뒤쪽에 위치하고 한 개 이상의 우 폐정맥의 환류 이상을 동반하고 있어 개심술시 정확한 교정을 요하므로 수술 전 또는 수술 중 정확한 해부학적 진단이 선행되어야 한다. 저자들은 부분 폐정맥 환류이상을 동반한 하정맥동형 심방중격결손 4예를 치험하였다. 1예는 25세 성인이었고 3예는 14개월 미만의 영아 및 소아였다. 4예 모두 심부전 때문에 수술했으며 이중 4개월의 영아는 긴급 수술이 필요했다. 진단을 위해 심초음파 및 도플러 검사와 심도자법을 시행하였다. 수술전 확진은 1예에서 가능했고 3예는 수술전 병변을 의심하고 수술시 확진되었다. 수술방법으로 이상 환류되는 폐정맥이 좌심방으로 환류 되도록 자가심막을 이용하여 심방중격결손을 폐쇄하였다. 4예 모두 수술 결과는 양호하였으며 초음파 검사의 추적에서 하대정맥과 폐정맥의 환류장애 소견은 없었다. 정확한 수술 교정을 위해 수술전 정확한 진단이 필수적이며, 수술전 폐정맥의 부분 환류이상과 심방중격 결손으로 진단된 환자에서는 수술중 하정맥동 결손의 여부를 확인하여 적절한 수술적 교정을 시행해야 할 것으로 사료된다.

• Journal of Chest Surgery
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• 제36권1호
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• pp.1-6
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• 2003

심장수술의 발달로 현재 심방중격결손증은 저 위험도의 안전한 수술로 인식되고 있다. 때문에 수술자체뿐만 아니라 미용적인 면에까지 관심의 대상이 되고 있다. 심방중격결손증의 폐쇄술에는 다양한 최소침습수술이 있겠으나 본원에서는 우전측부개흉술이 미용적인 면에서 우수하다고 판단되어 이를 정중흉골절개술과 비교 분석하였다. 대상 및 방법: 한양대학병원 흉부외과에서 1999년 1월부터 2002년 8월 까지 한명의 집도의에 의해 심방중격결손증으로 수술받은 환자 43명중 연속적으로 시행된 우전측부개흉술 15례(group A)와 동기간중 정중흉골절개술 15례(group B)를 임의적으로 추출하여 수술결과를 비교분석하였다. 결과: 환자의 평균체중은 group A 가 38.77$\pm$15.57kg 이었고 group B는 38.21$\pm$21.82kg 이었다. Group A 경우, 평균수술시간 197.6$\pm$61.40분, 평균체외순환시간 48.66$\pm$13.02분, 평균심실세동 혹은 대동맥 차단시간 30$\pm$11.64분이었고, Group B 경우, 평균수술시간 212.33$\pm$31.95분, 평균체외순환시간 55$\pm$12.10분, 평균심실세동 혹은 대동맥 차단시간 29.33$\pm$9.04분으로 서로간에 차이에 대한 통계적 유의성은 없었다. group A의 경우 수술 후 평균인공호흡기 사용시간은 3.78$\pm$0.78시간, 평균 중환자실 재실일수 1.2$\pm$0.47일, 평균 입원기간 10.20$\pm$1.08일 이었고, group B의 경우 수술후 평균 인공호흡기 사용시간은 5.95$\pm$3.73시간, 평균 중환자실 재실일수 1.41$\pm$0.61일, 평균입원기간 12.20$\pm$3.55일로 서로간에 차이에 대한 통계적 유의성이 없었다. 수술 후 1일간의 평균 출혈량은 group A의 경우 175.33$\pm$90.54cc이고, group B의 경우 352.33$\pm$239.43cc로 group A가 group B 에 비해 출혈량이 적은 것으로 나왔다(p.0.05). 합병증으로는 group B의 경우에서만 일시적인 2도 방실차단이 1례에서 있었으며 그외에 다른 합병증이나 사망률은 없었다. 결론: 우전측부개흥술은 정중흥골절개술과 비교 분석한 바 동일한 수술기구를 사용하면서도 미용적인 면에서 우수하며 수술 후 출혈량이 적었다(p〈0.05). 수술 난이도 면에서 우전측부개흉술이 수술시야가 좁아 어려웠으며 특히 대동맥 삽관에 주의가 필요하다.

• Journal of Chest Surgery
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• 제29권4호
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• pp.386-392
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• 1996

전북의대 흉부외과학교실에는 1984년 1월부터 1993년 6월까지 치험한 20세이상의 성인 심방중격 결손 60예를 대상으로 수술전후 결과를 분석하였다. 평균연령은40.2$\pm$ 12.5세였고남녀비는 1:2.3이었다 60예의 선천성 심방중격결손증에서 1차형 심 방중격이 2예, 2차형 심방중격이 58예이었고 수술은 1차공형은 모두 심막절편으로 폐쇄하였으며, 2 차공형은 78 %에서 단순봉합을 22 %에서는 심막절편으로 결손을 폐쇄하였다. 가장 흔한 증상은 운 동시 호흡곤란이었고 술전 NYHA 기능본류는 class 110예, class II 31예, class III 19예이 었고 class IV는 없었다. 술전 심전도 소견은 동정동 환자가 81%,동성 부정맥,동성서맥과 심방세동등의 부정맥이 19%, 우측편위가 30%, 우심실비대 32%에서 있었고, 전도장애의 소견중에 서는 불완전우각차단이 22% 로 가장 많았다. 술후 변화된 심전도 소견으로는 2예에서 심 방세동이, 1예에서 동성 빈맥 이 발생되 었 으나 모두 자연소실되거나 약물투여로 맥박조절이 가능하였으며 4예에서 우심실비대의 소실을, 2예 에서 우심방확장 소실이 관찰되 었다. 혈역학적 검사성적상 평균 Qp/qs는 술전, 술후 각각 3.75$\pm$1.21와 1.17 $\pm$ 0.11, 평균 Rp/Rs는 0.11 $\pm$ 0.09와 0.09 $\pm$ 0.12, 평균 수축기 폐동맥 압은 36.18 $\pm$ 14.05rnmHg와 24 $\pm$ 3.6이었다. 혈역학적 검사 성적을40세 이상과 40세 이하의 군으로 나눠 비교한 결과 40세 이상의 군에서 유의하게 수축기폐동 맥압이 높았으며 또 동정동 군자 부정맥 군의 비교에 있어서는 부정맥 군에서 평균연령과 평균수축 기 폐동맥 압이 높았다. 수술합병증은 부정맥 3예, 각각 1예의 기흥,혈흉,간염,탈모증이 있었으며 수술 사망예는 없었다. 평균 4년 7개월의 추적관찰기간중 45%에서 술후 중상의 호전을 보였다.

• Clinical and Experimental Pediatrics
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• 제59권1호
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• pp.16-23
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• 2016

Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

• Journal of Chest Surgery
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• 제21권4호
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• pp.649-655
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• 1988

One hundred and one patients with tetralogy of Fallot who were older than 16 years of age underwent a total correction of the anomaly between May, 1964 and July, 1987. This group comprised 14.9% of the 679 consecutive patients who had repair of the tetralogy at our institution during the same period. Of the 101 patients, 8 had a previous shunt procedure for palliation. The preoperative mean hemoglobin value was 16.9*1.0% and the mean systemic oxygen saturation, 84.4*0.9%. In 76 patients[75.2%], a type II ventricular septal defect was seen whereas in 14 patients[13.9%], the defect was type I. In 72 patients[71.3%], other cardiac anomalies were present which included patent foramen ovale in 37.6%, atrial septal defect in 8.99b, vegetations in 6.9%, right sided aortic arch in 5.9% and coronary artery anomaly in 5.0%. The right ventricular outflow obstruction was caused most commonly by combination of infundibular and valvular stenosis[74.3%], followed by isolated infundibular stenosis[19.8%] and valvular stenosis [5.9%] alone in order. The preoperative mean diameter of the pulmonary valve ring size was 10.2*0.5 mm in diameter. A transannular patch enlargement of the right ventricular outflow tract was performed in 28 patients and, in 12 a pericardial monocusp was utilized. Major anomalous aorto-pulmonary vessels were encountered in 5 patients which were detected before or during the operation. In 3 patients, they were ligated beforehand to control the flooding of the operative field. Postoperatively, the mean systolic pressure gradient between the right ventricle and the main pulmonary artery was 16.2*2.3 mmHg and the mean systolic pressure- ratio between the right and the left ventricle was 45.3*2.0%. Perioperative complications including bleeding in 8.9%, pleural effusion in 7.9%, dysrrhythmia in 4.9%, and residual VSD in 4.0%. Operative mortality was 8.9%. There has been no operative death in the recent 65 cases since 1981. There were 2 late deaths, 68 and 113 months after surgery. There were 2 late detachment of the VSD patch during the follow-up period. Of the 6 patients with patch detachment found during the postoperative period, 3 had subacute bacterial endocarditis before or after the operation indicating The serious nature of this complication. Two of these patients subsequently underwent a successful reoperation.