• Title/Summary/Keyword: tetralogy of fallot

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Repair of Coarctation (including tubular hypoplasia) in Infancy and Children (영아 및 소아 연령에서의 대동맥 교약증의 교정 수술)

  • 한재진
    • Journal of Chest Surgery
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    • v.23 no.3
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    • pp.474-481
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    • 1990
  • We have experienced 44 cases of coarctation of aorta in the age of infancy and children from April 1986 to September 1989 at Seoul National University Children`s hospital. Patients were thirty males and fourteen females, and their age ranged from one month to ten years[mean 23.84 $\pm$33.06 months] with thirty-two infant cases. In the infantile age, congestive heart failure was the most common chief complaint[18/32], and above that age, frequent upper respiratory infection was most common[8/12]. We experienced thirteen cases of isolated COA, twenty-two cases of COA with VSD, eight cases of COA with VSD, eight cases of COA with intracardiac complex anomalies and one case of COA with atrial septal defect. The associated intracardiac complex anomalies were three Taussig-Bing type double outlet right ventricle, one single ventricle, one transposition of great arteries, one atrioventricular septal defect, one hypoplastic aortic arch with left heart hypoplasia, and one Tetralogy of Fallot. Operative techniques of COA were twenty-three subclavian flap arterioplasty, 12 resection and end to end anastomosis, eight onlay patch angioplasty, and I direct angioplasty after resection of web. Among the cases with other cardiac anomalies, staged operation was done in twenty-nine patients, and single stage total correction was performed only in three patients. There were seven operative mortality[15.9%], all being in infantile age group, and among fourteen cases associated with large VSD[Qp/Qs>2.0, mean pulmonary arterial pressure>50mmHg], four patients were died, but there was no mortality in patients with small VSD. With above results, we are intended to discuss about the interval between staged operation, the fate of VSD after coarctoplasty in case of COA with VSD, causes of death, complications etc.

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A Case of Isolated Congenital Absence of Right Pulmonary Artery (선천성 우측 폐동맥 형성부전증 1예)

  • La, Sung Soo;Kim, So Mi;Kim, Doh Hyung
    • Tuberculosis and Respiratory Diseases
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    • v.65 no.5
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    • pp.430-434
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    • 2008
  • Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

Diagnostic Validity of RI Angiocardiography in Cardiac Diseases (심질환(心疾患)의 RI angiocardiography에 관한 진단적(診斷的) 가치(價値))

  • Kim, Kwan-Yop;Koh, Chang-Soon;Lee, Mun-Ho
    • The Korean Journal of Nuclear Medicine
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    • v.6 no.2
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    • pp.21-39
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    • 1972
  • The employment of gamma-ray scintillation camera with the use of short-lived radioisotopic pharmaceuticals, sucu as $^{99m}Tc$ sodium pertechnetate, have enabled us to perform RI (radioisotopic) angiocardiography. Although conventional cardiac catheterization or angiocardiography using contrast media have been an important diagnostic tool, they may carry some risks or serious complications. The author investigated on RI angiocardiography in twelve normal and twenty five patients with cardiovascular diseases in an effort to evaluate its diagnostic value. The results obtained with this study are as follows; 1. In normal subjects, the scintillation camera transit time of arm-to-right heart was found to be $2.1{\pm}0.67$ seconds, right heart-to-lung $1.5{\pm}0.40$ seconds and lung-to-left heart $3.5{\pm}0.86$ seconds. 2. Transformation or displacement of the heart and the great vessel are easily discriminated by RI angiocardiography. Both in the cases with tricuspid atresia and tetralogy of Fallot, ventricular septal defects are well recongnized by sequential RI angiocardiography. 3. It is also helpful in determinining the site and extent of obstruction, and estimatiing the postoperative course both in the cases with superior vena cava syndrome and pulmonary stenosis. 4. Pericarditis with effusion is readilly diagnosed by RI angiocardiogram showing characteristic "dead space" between intracardiac and intrapulmonary radioactivity. 5. It was found that the diagnostic accuracy of this study was 78.4%. It is concluded that above results obtained are useful and accurate enough for the diagnostic screening methode for clinical practice.

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Open Heart Surgery During The First 12 Months Of Life (유아기의 개심술14례 보고)

  • Ahn, Kyuk;Suh, Kyung-Phill
    • Journal of Chest Surgery
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    • v.14 no.4
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    • pp.381-387
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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.

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Right Pulmonary Artery Agenesis - A case report - (우측 폐동맥 형성부전증 - 1예 보고 -)

  • Kim, Hyuck;Wee, Jang-Seop;Kim, Young-Hak;Chung, Won-Sang;Nam, Seung-Hyuk;Kang, Jeong-Ho
    • Journal of Chest Surgery
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    • v.40 no.1 s.270
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    • pp.52-55
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    • 2007
  • Unilateral pulmonary artery agenesis is a rare congenital malformation usually associated with other cardiovascular anomaly such as Tetralogy of Fallot. Isolated pulmonary artery agenesis is very rare, and usually asymptomatic. It is usually highly suspected by routine chest X-ray, and associated symptoms are hemoptysis, blood tinged sputum, repeated pulmonary infection, and dyspnea on exertion. We have recently experienced the right pulmonary artery agenesis in 27 year-old male patient, complaining of minimal hemoptysis and sustained blood tinged sputum. He was successfully treated by right pneumonectomy, so we report this case with the review of associated literature.

A Quantitative Analysis of Nonlinearity Changes of 24 hour Heart Rate Variability of TOF Children Group and Normal Children Group (TOF 소아 집단과 정상 소아 집단의 24 시간 심박동수 변동량의 비선형성 변화에 대한 정량적 분석)

  • Lee, J.M.;Noh, J.I.;Park, K.S.
    • Proceedings of the KOSOMBE Conference
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    • v.1997 no.11
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    • pp.451-454
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    • 1997
  • It has been reported that sudden cardiac death and ventricular tachycardia occur after treatment of tetralogy of fallot(TOF). It is regarded that ventricular arrythmia is the main source or the sudden cardiac death, but it is not verified. It is likely that TOF has effect on the heart rate variability because of the ventricular arrythmia. We study how complex and periodic heart rate dynamics change in the normal children (n=13) and TOF children (n=13) throughout 24 hours. We recorded 24-hour holter ECG, and segmented each ECG data into 1 hour length. We analyze each HR time series, and quantify the overall complexity of each HR time series by its correlation dimension. We also calculate the power spectrum of HR, and obtain low-frequency component (0.03-0.15Hz) and high-frequency component (0.15-0.4Hz). We compare the results between normal and TOF groups throughout 24 hours. TOF group have lower correlation dimension ($4.055{\pm}0.4134$ vs. $4.9310{\pm}0.2054$, p<0.05) than the normal group, even though there are no significant differences in the low($0.9864{\pm}0.5598$ vs. $1.5560{\pm}0.8325$, p<0.05) and high($1.1168{\pm}0.1.1448$ vs. $0.9271{\pm}0.6528$, p<0.05) frequency components. It can be concluded that HR time series of TOF group are more regular than that of normal group, and that correlation dimension reveals a nonlinear characteristics of HR time series which is not determined in the frequency domain.

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Open Heart Correction Of Ebstein`S Anomaly: A Report Of 8 Cases (Ebstein`s 심기형의 개심수술 8예)

  • 김삼현
    • Journal of Chest Surgery
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    • v.14 no.4
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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.

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Systemic-Pulmonary Shunts Using Microporous Polytetrafluoroethylene Prosthesis [Early and Late Results] (Polytetrafluoroethylene 인조혈관을 이용한 체-폐동맥 단락술의 조기성적 및 원격 성적)

  • 장병철
    • Journal of Chest Surgery
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    • v.19 no.1
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    • pp.50-57
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    • 1986
  • Sixty-nine patients with various types of cyanotic congenital heart disease underwent systemic-pulmonary artery shunts with a microporous polytetrafluoroethylene [PTFE] prosthesis between 1979 and 1985. Their ages ranged from 2 months to 39 years [mean$\pm$SD: 5.2$\pm$7.4, median: 3.3 years]. Diagnosis included the following: Tetralogy of Fallot, 45: Double outlet right or left ventricle, 11: Single ventricle, .5: Transposition of great vessels, 4: Tricuspid atresia, 3 and Pulmonary atresia with intact ventricular septum, 1. Forty-eight patients had subclavian-pulmonary artery anastomosis, 12 patients aorta-right pulmonary artery anastomosis, 6 patients aorta-main pulmonary artery anastomosis, and 3 patients descending aorta-pulmonary artery anastomosis. The PTEE graft of 3 mm in diameter was used in 1, 4 mm in 29, 5 mm in 35 and 6 mm in 4 patients. Ten patients were died within 30 days after operation [mortality rate: 14.5%]. Among them, 6 patients were operated in urgency due to cardiac arrest or severe anoxic spell after cardiac catheterization, and so surgical mortality of elective operation is 9.5%. The 59 survivors showed improvement of the arterial oxygen saturation [65.4% - 9.8%] and hemoglobin [18.8 gm/dl - 16.0 gm/dl] values [V<0.01]. The follow up period ranged from 1 month to 67 months, [752 patient-months] and during this periods there were 4 late shunt failures after 3 months postoperatively with 4 mm graft, and 2 with 5 mm graft. The over-all patency rate of 4 mm PTFE was 85.9$\pm$9.2% [SEM] in 12 months and 40.9$\pm$22.5% in 24 months. The over-all patency rate of 5 mm PTFE was 87.5$\pm$9.6% in 12 months and 58.3$\pm$24.6% in 36 months. The lowest systolic pressure in death group was 64.9$\pm$15.0 mmHg and in survival group, 86.4$\pm$12.1 mmHg [P<0.001]. We think that the PTFE graft is useful in palliative shunt operation, but the effectiveness of the 4 mm PTFE graft may be limited. The blood pressure also may play an important role in patency of Prosthesis.

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Right Pulmonary Artery Agenesis -A Case Report- (우측 폐동맥 형성부전증 -수술치험 1례-)

  • Sin, Dong-Geun;Kim, Min-Ho;Kim, Gong-Su
    • Journal of Chest Surgery
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    • v.30 no.1
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    • pp.108-111
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    • 1997
  • Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

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Pulmonary Function Following Open Heart Surgery -early and late postoperative changes- (개심술후 폐기능 -수술직후 및 장기간의 추이에 대하여-)

  • 이성행
    • Journal of Chest Surgery
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    • v.13 no.4
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    • pp.364-374
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    • 1980
  • Twenty-two patients were selected for evaluation of pre-and postoperative pulmonary function. These patients were performed open cardiac surgery with the extracorporeal circulation from March 1979 to July 1980 at the Department of Thoracic and Cardiovascular Surgery, Kyungbook National University Hospital. Patients were classified with ventricular septal defect 5 cases, atrial septal defect 5 cases, tetralogy of Fallot 5 cases, mitral stenosis 4 cases, rupture of aneurysm of sinus Valsalva 1 case, left atrial myxoma I case, and aortic insufficiency 1 case. The pulmonary function tests were performed and listed: [1] respiratory rate, tidal volume [TV], and minute volume[MV], [2] forced vital capacity [FVC] and forced expiratory volume[FEV 0.5 & FEV 1.0], [3] forced expiratory flow [FEF 200-1200 ml & FEF 25-75%]. [4] Maximal voluntary ventilation [MVV], [5] residual volume [RV] and functional residual capacity[FRC], measured by a helium dilution technique. Respiratory rate increased during the early postoperative days and tidal volume decreased significantly. These values returned to the preoperative levels after postoperative 5-6 days. Minute volume decreased slightly, but essentially unchanged. Preoperative mean values of the forced vital capacity, functional residual capacity and total lung capacity decreased [63.2%, 87.2% & 77.3% predicted, respectively], and early postoperatively these values decreased further [19.6%, 76.0% & 38.0% predicted], but later progressively increased to the preoperative levels. In residual volume, there was no decline in the preoperative mean values [100.9% predicted] and postoperatively the value rather increased [106.3-161.7% predicted]. Forced expiratory volume [FEV 0.5 & FEV 1.0] and forced expiratory flow [FEF 200-1200 ml & FEF 25-75%] also revealed significant declines in the early postoperative period. There was no significant difference in values of the spirometric pulmonary function tests, such as FEF 1.O and FEF 25-75% between successful weaning group [17 cases] extubated within 24 hrs post-operatively and unsuccessful weaning group [5 cases] extubated beyond 24 hrs. Static compliance and airway resistance measured for the two cases during assisted ventilation, however, any information was not obtained. Long term follow-up pulmonary function studies were carried out for 8 cases in 9 months post-operatively. All of the results returned to the pre-operative or to normal predicted levels except FVC, FEV 1.0, and FEF 25-75% those showed minimal declines compared to the pre-operative figures.

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