• Clinical and Experimental Pediatrics
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• 제57권1호
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• pp.19-25
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• 2014

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

• Journal of Chest Surgery
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• 제42권6호
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• pp.770-773
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• 2009

심장횡문근종은 영, 유아나 소아기에서 가장 발생 빈도가 높은 원발성 심장종양으로서, 다발성인 경우 빈번히 결절성 경화증을 동반한다. 저자들은 심장 횡문근종 및 결절성 경화증과 동반된 활로씨 사징증을 가진 환아에서 신생아기에 전신-폐 단락술을 시행하고 횡문근종이 자연적 소멸된 후 2세에 완전 교정술을 시행하여 양호한 결과를 얻었기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제24권2호
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• pp.115-122
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• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• Journal of Chest Surgery
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• 제50권3호
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• pp.215-219
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• 2017

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the rig ht lung, and rig ht lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the rig ht pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.

• Journal of Chest Surgery
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• 제23권2호
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Journal of Chest Surgery
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• 제24권2호
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• pp.153-160
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• 1991

Tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 91 of 250 patients treated surgically [including redo operations] between April 1986 and December 1989. Their age ranged from 6 months to 14 years [mean 39.7 months]. Associated cardiovascular anomalies were right aortic arch [n=22], ASD [n=12], PDA [n=5], persistent left SVC [n=5], and others [n=6]. PA index was measured pre-operatively since 1987 to estimate pulmonary artery size and safe total correction[mean 289$\pm$110mm2/BSA]. Eight patients received previous shunt take down procedure concomitantly. Pulmonary arteriotomy was extended through small pulmonary annulus to a minimal distance upon the right ventricular infundibulum and transannular patch was applied in 38 patients [41.3%], in 31 of them monocuspid patch was utilized. pRV/LV was measured at operation room in 77 patients [mean 0.58$\pm$0.36]. Operative mortality was 6.6% [6/91]. The causes of death were low cardiac output [n=5], arrhythmia[n=1] and respiratory failure [n=1]. At follow-up between 12 months and 57 months [mean 30.8 months] most patients were in New York Heart Association class I without cardiac medication. There was no late death, but reoperations were required in 3 patients to relieve residual right ventricular outflow obstruction. Thus successful repair of tetralogy of Fallot can be accomplished in most patients including infants by transatrial-transpulmonary approach and the better result can be anticipated with respect to postoperative right ventricular function and arrhythmia than the conventional transventricular approach.

• Journal of Chest Surgery
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• 제16권3호
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• pp.337-341
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• 1983

The experience with operative treatment for total correction of Tetralogy of Fallot at the department of Thoracic and Cardiovascular Surgery, Korea University Hospital from January, 1977, through April, 1983 was reviewed. Of the 29 patients reviewed, male to female occurrence ratio was 22:7 Type of V.S.D. was type II in 26 cases[90%] and total conus defect in 3 cases[10%]. Average size of V.S.D. was 19 mm. Type of Right ventricular outflow tract stenosis was highest frequency with pulmonary valvular and infundibular stenosis combined type in 21 cases[72%], and there were 8 deaths In this group. Type of R.V.O.T. reconstruction contains 2 cases of infundibulectomy only, 1 case of infundibulectomy with valvular commissurotomy, 2 cases of pericardial patch, 23 Cases of pericardial patch with Teflon or Dacron felt reinforced and 1 case of pulmonary valved conduit reconstruction. Operative mortality was higher in outflow patch through pulmonary valve ring. Overall mortality was 31%. Major causes of death and postoperative complications were low output syndrome, complete A-V block acute renal failure, ventricular fibrillation, bleeding brain abscess, and sudden cardiac arrest.

• Journal of Chest Surgery
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• 제35권6호
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• pp.460-462
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• 2002

본 저자들은 활로씨 4징증을 동반한 완전방실중격결손 환자에서 완전교정수술을 시행하였다. 술전 검사로 심초음파, 심도자검사, 심조영술을 시행하였다. 수술은 체외순환을 시행하여 두 개의 첩제를 이용하여 방실중격결손을 폐쇄하였고, 우심실절개와 폐동맥판교련술을 통해 우심실 누두부 절제술과 경판륜 첩제를 사용하여 우심실유출로 확장술을 시행하였다. 술후 시행한 초음파 검사상 경도의 승모판과 삼첨판의 폐쇄부전이 나타났으나 혈역학적 이상소견은 없었다.

• Journal of Chest Surgery
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• 제29권11호
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• pp.1197-1201
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• 1996

인제대학교 의과대학 서울백병원 흉부외과학교실에서는 1987년 10월부터 1995년 12월까지 20세 이상의 성인 팔로사징후 9례에서 완전교정술을 시행하였다. 환자는 남자 5례, 여자 4례로 연령은 22세에서 42세까지였고 평균 29.6세였다. NYHA에 의한 기능적 분류는 Class II가 3례, Class III가 6례였고, 혈색소치는 10.8 ∼ 20.7 gm/㎗(평균 15.6 gm/㎗)였다. 술전임상 증상 및 징후는 청색증 8례, 운동시 호흡곤란 6례, 곤봉수지 5례, 잦은 상기도 감염 3례 였다. 폐동맥판 및 누두부협착이 모든 예에서 있었고, 2례에서는 좌측폐동맥에 발육부전이 있었다. 우심실유출로 협착에 대한 수술은 7례에서는 누두부절제술 및 판막절개술후 누두부에만 Goretex 첩포를 대어 확장술을 하였고, 2례에서는 누두부절제술과 판막절개술후 폐동맥 판막륜이 작아 이를 절개하여 주폐동맥까지 Goretex 첩포를 대어 확장하였다. 좌측 폐동맥에 발육부전이 있는 2례에서는 자가심낭편을 이용하여 폐동맥성형술을 하였다. 술후 사망환자는 없었고 합병증은 6례에서 발생하였는데 출혈이 가장 많아 5례에서 있었고 저심박출증 4례(44.4%)였다. 1례에서 일차수술 3개월후 잔존 심실중격결손과 삼첨판폐쇄부전이 있어 재수술을 하였다. 평균 추적관찰기간은 평균 25개월(범위, 11∼77개월)이었다. 술후 모든 환자는 NYHA class I이었다. 따라서 팔로사징후에서 나이가 들었다는 자체가 수술의 금기는 아니며, 성인 팔로사징후는 사망률도 낮으므로 완전교정이 가능하다고 사료된다.

• Journal of Chest Surgery
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• 제16권1호
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• pp.40-48
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• 1983

Tetralogy of Fallot is characterized by the interventricular septal defect associated with obstruction of the right ventricular outflow. The importance of the latter anatomic malformation was clinically evaluated according to the angiographic measurements of the pulmonary arteries along with the pulmonary valve annulus. Seventy of a total 76 patients operated on during a whole year of 1981 were the patients for clinical evaluation. Fifteen patients died within 1 month after operation with the operative mortality of 21.4%. The young age and the severity of pulmonary arterial hypoplasia were ones among the surgical risk factors at a total corrective surgery of tetralogy. Preoperative angiographic measurements of the pulmonary arteries to speculate the expected postoperative ratio between the left ventricular and the right ventricular pressures were retrospectively calculated according to the formula. The predicted values of P RV/LV greater than 0.5 carried apparenliy higher complication and mortality rates than the group of P RV/LV less then 0.5. The selection of the candidates for either a total correction or the staged operation In tetralogy of Fallot can be evaluated on the preoperative angiographic measurements and the expected Improvements of the clinical results were discussed.