• 제목/요약/키워드: tetralogy of fallot

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Fallot 사징증(四徵症) 외과적(外科的) 완전교정(完全矯正)에 대한 임상적(臨床的) 고찰(考察) (A Review of Total Correction in 48 Cases of Tetralogy of Fallot)

  • 서경필
    • Journal of Chest Surgery
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    • 제9권2호
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    • pp.198-206
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    • 1976
  • A Total of Forty eight patients underwent open-heart surgery for correction of tetralogy of Fallot at the Seoul National University Hospital from January 1974 to October 1976, with an overall survival rate of 77 per cent. Operative mortality varied according to severity of the lesion, age of the patient, nature of previous surgical treatment and presence or absence of an outflow tract patch across the pulmonary valve ring. Eleven patients died in the early postoperative period and thirty seven patients were discharged from the hospital alive. A patch of the right ventricular outflow tract and pulmonary annulus was required to relieve pulmonic stenosis in 24 patients. There were 10 deaths in this group (42%) as compared to 1 death in the group of 24 patients who were corrected without a patch. Operative mortality was especially higher when an inlay patch was placed across the pulmonary valve ring. This may be related to the possibly greater anatomic severity of these cases and to the longer operating time when a patch was used. The electrocardiogram showed right ventricular hypertrophy in 35 cyanotic patients. Intraventricular conduction was normal in 34 patients before operation. It was normal postoperatively in only 5 of 34 patients in this group who survived surgery. Complete right bundle branch block appeared at operation in 21 patients, and 8 patients developed incomplete right bundle branch block. Major causes of death were progressive cardiac failure (4), Complete atrioventricular dissociation (3), bleeding (2), cardiac tamponade (1), and sudden cardiac arrest (1)

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Fallot 4징증의 근치수술후 예후에 영향을 미치는 요소 (Factors influencing the prognosis after total correction of tetralogy of Fallot)

  • 민용일;오봉석;이동준
    • Journal of Chest Surgery
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    • 제20권1호
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    • pp.71-80
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    • 1987
  • Twenty six patients were operated a total correction of tetralogy of Fallot between Jan., 1984 and July, 1985 at the Dept. of thoracic and cardiovascular surgery, Chonnam University Medical School, and a comparison between the survived group [n = 18] and the dead group [n = B was performed to detect factors influencing laboratory data, cineangiographic findings, operative findings and methods, and pump time. Following results were obtained, 1. There was no significant difference between two groups in the preoperative P.O2 and hematocrit level. 2. The size of the interventricular defect was not related to the operative mortality. 3. There was a significant difference in mortality rate between combined type of stenosis of pulmonary artery, valve and infundibulum and other types of right ventricular outflow tract stenosis. 4. There was a significant difference in mortality rate between the transannular patch reconstruction and other types of operative procedure. 5. There was no significant difference between two groups in total bypass time and aortic time. 6. There was no significant difference between two groups in left ventricular end diastolic volume and right ventricular end diastolic volume. 7. The operative mortality was related to the ratio of the diameter of the pulmonary valve annulus or each pulmonary artery to the ascending or descending aorta in cineangiographic findings, but there was no statistical significance of which probably is due to the lack of the total number of patients.

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Fallot 4 징증에서 변형 Blalock Taussig 수술후 폐동맥성장에 관한 연구 (A Study on Growth of Pulmonary Artery after Modified Blalock-Taussig Shunt in Tetralogy of Fallot)

  • 양태봉;오봉석;이동준
    • Journal of Chest Surgery
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    • 제21권1호
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    • pp.10-16
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    • 1988
  • Ten patients with tetralogy of Fallot were studied angiocardiographically before a modified Blalock-Taussig shunt and again 25*3.2 months after the previous shunt. All of ten patients had patent previous shunt at the time of follow up examination. Pre-and postoperative diameters of left and right pulmonary artery and descending aorta were measured and pulmonary artery index [the sum of the crossectional areas of the right and left pulmonary arteries standardized by the body surface area] was calculated. The ratio of mean diameter of left and right pulmonary arteries to the diameter of the descending aorta [LPA+RPA/2xDA] was increased postoperatively by 0.20*0.068 [p=0.020]. Mean PAI [pulmonary artery index] increased from 283.8*178.4 mm/m2 BSA to 345.8~144.5 mm/m2 BSA after shunt operation [p=0.019]. This results suggested that the modified Blalock-Taussig shunt was effective to help growth of the pulmonary arteries in most cases of the study populations but the ones with the PAI>233mm*/m* BSA appeared less benefited by Blalock Taussig shunt. Calculation of PAI could be an aid to making a decision whether to perform a one stage corrective surgical procedure or a palliative shunt procedure in the patient with small pulmonary arteries.

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수술 후 성인 Fallot 4징 환자의 임상적 고찰 (Follow-up in Adult after Total Repair of Tetralogy of Fallot)

  • 장기영;김선영;문주령;허준;강이석;박승우;전태국;박표원;이흥재
    • Clinical and Experimental Pediatrics
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    • 제46권7호
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    • pp.661-667
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    • 2003
  • 목 적 : Fallot 4징 환자에 대한 수술 및 수술 후 집중 치료의 발달로 생존율이 향상되면서 교정 수술을 받은 Fallot 4징 환자의 연령이 점차 증가하였고, 이에 따라 장기 생존에 따른 여러가지 문제점들이 알려지게 되었다. 저자들은 완전 교정 수술을 받은 성인 Fallot 4징 환자에서 나타나는 주요 임상적인 문제점들을 알아보고자 하였다. 방 법 : 1994년 5월부터 2000년 7월까지 삼성서울병원 성인선천성심장병클리닉(GUCH Clinic)에 등록된 Fallot 4징 환자는 30명으로, 내원 당시 자연 생존자는 6명, 수술 후 생존자는 24명이었다, 이들중 본 조사 당시까지 완전 교정 수술을 받은 환자 28명을 대상으로 임상 기록, 심전도, 심장 초음파, 심도자 검사, 수술 기록 등을 후향적으로 조사하여 주요 임상 문제점을 알아보았다. 결 과 : 1) 본 조사 당시 평균 연령은 30.8세(16-53세)였고, 교정 수술시 나이는 15.8세(2-49세)였다. 남녀 비는 1.3 : 1이었다. 2) 완전 교정 수술 후 임상적으로 유의한 문제점들은 부정맥(11례), 폐동맥 역류(8례), 좌폐동맥 협착(6례), 잔존 심실 중격 결손(6례), 승모판막 역류(5례), 삼첨판막 역류(4례), 우심실 유출로 협착(3례), 우심실 유출로 낭성 확장(3례), 대동맥판막 역류(3례), 심내막염(1례) 및 단백 소실 장증(1례) 등이었다. 3) 부정맥이 있었던 환자군(Group I, 11명)과 부정맥이 없었던 군(group II, 17명)으로 나누어 비교하여 보았을 때, 수술 시 연령이 낮을수록 수술 후 부정맥이 적게 관찰되었고(P<0.05), 부정맥 군이 부정맥이 없었던 군에 비해 흉부 방사선 소견상 심.흉 비 0.55 이상의 심비대가 유의하게 많았다(P<0.05). 심전도에서 QRS폭이 넓을수록 심비대가 많았으며(P<0.05), 수술 후 심비대가 있는 군(심/흉 비>0.55)과 심비대가 없는 군으로 나누어 비교해 보았을 때는 심비대가 있는 군에서 QRS폭이 넓었고 (P<0.05), 의미 있는 폐동맥 역류와 삼첨판막 역류가 많이 관찰되었다(P<0.05). 4) 재수술은 총 28례 중 8례에서 시행되었다. 재수술의 원인으로, 잔존 심실 중격 결손만 있었던 경우가 3례, 우심실 유출로 협착이 동반된 잔존 심실 중격 결손이 1례, 우심실 유출로 협착과 폐동맥 역류가 동반된 잔존 심실 중격 결손이 1례, 우심실 유출로 협착, 감염성 심내막염으로 인한 삼첨판막 우종 및 잔존 심실 중격 결손이 1례, 좌폐동맥 협착이 1례, 우폐동맥 협착(추정)이 1례 있었다. 결 론 : Fallot 4징은 교정 수술 후 대부분의 환자가 별 문제없이 정상적인 생활이 가능하지만 본 조사에서처럼 여러 가지 문제점들-부정맥, 폐동맥 역류, 잔존 심실 중격 결손, 우심실 유출로 협착, 폐동맥분지 협착, 감염성 심내막염 등-이 발생할 수 있으므로 장기적인 예후를 좋게 하기 위해서는 정기적이고도 지속적인 관찰과 조기 교정이 필요하다고 생각된다.

Successful Opening of Ductus Arteriosus with Milrinone in a Newborn with Tetralogy of Fallot and Pulmonary Atresia

  • Won, Chong-Bock;Kim, Min-Young;Choi, Duk-Young;Cho, Hye-Jung;Shim, So-Yeon;Son, Dong-Woo
    • Neonatal Medicine
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    • 제18권2호
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    • pp.365-369
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    • 2011
  • 폐동맥폐쇄가 동반된 팔로네증은 팔로네증 중에 가장 심한 형태이다. 이런 종류의 선천성 심질환에서 폐혈액 순환을 지속하기 위하여 동맥관 개존을 유지하는 것은 생명을 구하는 매우 중요한 치료이다. Milrinone은 선택적 인산이에스테르효소 3 억제제이며 강력한 혈관 확장제로 알려져 있다. 저자들은 폐동맥 폐쇄가 동반된 팔로네증 신생아에서 폐순환을 지속시키기 위하여 동맥관 개방을 유지하는 치료로 milrinone을 성공적으로 사용한 증례를 보고한다. Milrinone은 동맥관 개방효과뿐 아니라, 심근수축 촉진, 이완 촉진, 폐혈관 확장 효과가 있으며 부작용은 심하지 않아 더 이로운 치료방법이 될 가능성이 있다. Milrinone의 치료효과를 밝히기 위하여 더 많은 치료증례 조사와 타 약제와의 비교 연구 등이 필요하다.

우심실유출로협착에 대한 심낭 Patch Graft 에 관한 임상적 연구 (A study on Hemodynamic Effect of Pericardial Patch Graft for Stenosis of Right Ventricle Outflow Tract)

  • 박정수;지행옥;김근호
    • Journal of Chest Surgery
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    • 제15권1호
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    • pp.61-66
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    • 1982
  • The present study was performed to evaluate hemodynamic effects on the pericardial patch graft for stenosis of right ventricle outflow tract in 19 patients of tetralogy of Fallot. The stenosis of right ventricle outflow tract was associated with or without pulmonary annular nar-rowing, pulmonary valvular stenosis, and hypoplastic narrowing of pulmonary artery. Total correction of tetralogy of Fallot was performed under cardiopulmonary bypass with moderate hypothermia and cardioplegic cardiac arrest. Ventricular septal defects were closed with Teflon patch graft. The chamber pressures in the heart were measured before and after a total correction of tetralogy of Fallot. The data of pressure measurement and the results of postoperative observation of pericardial patch were as followings: 1. Systolic and diastolic pressure of right ventricle was decreased after operation from $96.0{\pm}14.7/10.0{\pm}14.4mmHg$ to $61.0{\pm}13.1/8.0{\pm}9.3mmHg$. 2. Systolic and diastolic pressure of pulmonary artery was increased after operation from $18.0{\pm}5.6/10.0{\pm}5.5mmHg$ to $31.0{\pm}10.7/14.0{\pm}4.9mmHg$. 3. Preoperative pressure gradient between right ventricle and pulmonary artery was decreased immediately after operation from 78.0mmHg to 30.0mmHg. 4. It was observed that excellent widening effects of right ventricle outflow tract was resulted from pericardial patch graft. 5. No postoperative bleeding from pericardial patch graft was observed. 6. Aneurysm formation of pericardial patch was not be observed during 1 to 6 years postoperative periods.

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Repair of Tetralogy of Fallot in Infancy via the Atrioventricular Approach

  • Bigdelian, Hamid;Sedighi, Mohsen
    • Journal of Chest Surgery
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    • 제49권1호
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    • pp.9-14
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    • 2016
  • Background: Tetralogy of Fallot (TOF) is a well-recognized congenital heart disease. Despite improvements in the outcomes of surgical repair, the optimal timing of surgery and type of surgical management of patients with TOF remains controversial. The purpose of this study was to assess outcomes following the repair of TOF in infants depending on the surgical procedure used. Methods: This study involved the retrospective review of 120 patients who underwent TOF repair between 2010 and 2013. Patients were divided into three groups depending on the surgical procedure that they underwent. Corrective surgery was done via the transventricular approach (n=40), the transatrial approach (n=40), or a combined atrioventricular approach (n=40). Demographic data and the outcomes of the surgical procedures were compared among the groups. Results: In the atrioventricular group, the incidence of the following complications was found to be significantly lower than in the other groups: complete heart block (p=0.034), right ventricular failure (p=0.027) and mediastinal bleeding (p=0.007). Patients in the atrioventricular group had a better postoperative right ventricular ejection fraction (p=0.001). No statistically significant differences were observed among the three surgical groups in the occurrence of tachycardia, renal failure, and tricuspid incompetence. The one-year survival rates in the three groups were 95%, 90%, and 97.5%, respectively (p=0.395). Conclusion: Combined atrioventricular repair of TOF in infancy can be safely performed, with acceptable surgical risk, a low incidence of reoperation, good ventricular function outcomes, and an excellent survival rate.

Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair

  • Yoo, Byung Won;Park, Han Ki
    • Clinical and Experimental Pediatrics
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    • 제56권6호
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    • pp.235-241
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    • 2013
  • Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.

Predictors of Intensive Care Unit Morbidity and Midterm Follow-up after Primary Repair of Tetralogy of Fallot

  • Egbe, Alexander C.;Nguyen, Khanh;Mittnacht, Alexander J.C.;Joashi, Umesh
    • Journal of Chest Surgery
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    • 제47권3호
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    • pp.211-219
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    • 2014
  • Background: Our objectives were to review our institutional early and midterm experience with primary tetralogy of Fallot (TOF) repair, and identify predictors of intensive care unit (ICU) morbidity. Methods: We analyzed perioperative and midterm follow-up data for all cases of primary TOF repair from 2001 to 2012. The primary endpoint was early mortality and morbidity, and the secondary endpoint was survival and functional status at follow-up. Results: Ninety-seven patients underwent primary repair. The median age was 4.9 months (range, 1 to 9 months), and the median weight was 5.3 kg (range, 3.1 to 9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median length of ICU stay was 6 days (range, 2 to 21 days), and the median duration of mechanical ventilation was 19 hours (range, 0 to 136 hours). By multiple regression analysis, age and weight were independent predictors of the length of ICU stay, while the surgical era was an independent predictor of the duration of mechanical ventilation. At the 8-year follow-up, freedom from death and re-intervention was 97% and 90%, respectively. Conclusion: Primary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.