• Clinical and Experimental Pediatrics
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• 제46권7호
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• pp.627-634
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• 2003

• 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.

• 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.

• 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.

• 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.

• 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.

• 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.

• Journal of Chest Surgery
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• 제11권3호
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• pp.326-332
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• 1978

There appears some conduction defects frequently after total correction of Tetralogy of Fallot. Common defect is right bundle branch block due to surgical intervention. We experienced complete A-V block which occured 3 months later after total correction of Tetralogy of Fallot in a 8 year old boy. The patient was completely free of any A-V block after the operation for 3 months, and sudden onset of A-V block with coupled premature ventricular contractions resulted him in shock state during the attack of severe bronchopneumonia for 4 days prior to the second visit. Emergency implantation of Cordis demand type temporary pacemaker was necessary to control the complete heart block with bradyarrhythmia and frequent ventricular fibrillation. Permanent cardiac pacemaker was implanted two weeks later as indicated with Cordis Stanicor lambda demand pacemaker, and the patient was discharged uneventfully on the 8th post implantation day with the heart rate of 72/min. Another 3 months after the implantation, the patient was transported to this hospital as dead on arrival after an accidental fall from a 2 meter height, and all possible cardiopulmonary resuscitation was performed for 60 minutes at the emergency room in vain. Autopsy was done to find out the cause of sudden death and the etiology of complete heart block. Microscopic focal infarctions with scar formation were noted along the course of conduction system in the interventricular septum, which might be the main cause of complete heart block during the attack of severe bronchopneumonia complicated with acute bacterial endocarditis. The tip of the pacemaker wire was slipped from the granulation scar at the apex of the right ventricular cavity, and this might be the direct cause of pacing failure and death.

• Journal of Chest Surgery
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• 제50권2호
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• pp.71-77
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• 2017

Background: Cardiac computed tomography (CT) has emerged as an alternative to magnetic resonance imaging (MRI) for ventricular volumetry. However, the clinical use of cardiac CT requires external validation. Methods: Both cardiac CT and MRI were performed prior to pulmonary valve implantation (PVI) in 11 patients (median age, 19 years) who had undergone total correction of tetralogy of Fallot during infancy. The simplified contouring method (MRI) and semiautomatic 3-dimensional region-growing method (CT) were used to measure ventricular volumes. Results: All volumetric indices measured by CT and MRI generally correlated well with each other, except for the left ventricular end-systolic volume index (LV-ESVI), which showed the following correlations with the other indices: the right ventricular end-diastolic volume index (RV-EDVI) (r=0.88, p<0.001), the right ventricular end-systolic volume index (RV-ESVI) (r=0.84, p=0.001), the left ventricular end-diastolic volume index (LV-EDVI) (r=0.90, p=0.001), and the LV-ESVI (r=0.55, p=0.079). While the EDVIs measured by CT were significantly larger than those measured by MRI (median RV-EDVI: $197mL/m^2$ vs. $175mL/m^2$, p=0.008; median LV-EDVI: $94mL/m^2$ vs. $92mL/m^2$, p=0.026), no significant differences were found for the RV-ESVI or LV-ESVI. Conclusion: The EDVIs measured by cardiac CT were greater than those measured by MRI, whereas the ESVIs measured by CT and MRI were comparable. The volumetric characteristics of these 2 diagnostic modalities should be taken into account when indications for late PVI after tetralogy of Fallot repair are assessed.

• Journal of Yeungnam Medical Science
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• 제34권2호
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• pp.238-241
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• 2017

Background: We investigated the difference in right ventricle (RV) volume and ejection fraction (EF) according to the pulmonary valve (PV) annular extension technique during Tetralogy of Fallot (TOF) total correction. Methods: We divided patients who underwent the procedure from 1993 to 2003 into two groups according to PV extension technique (group I: PV annular extension, group II: no PV annular extension) during TOF total correction. We then analyzed the three segmental (RV inlet, trabecular and outlet) and whole RV volume and EF by cardiac magnetic resonance imaging (MRI). Results: Fourteen patients were included in this study (group I: 10 patients, group II: four patients; male: nine patients, female: five patients). Cardiac MRI was conducted after a 16.1 years TOF total correction follow-up period. There was no statistical difference in RV segmental volume index or EF between groups (all p>0.05). Moreover, the total RV volume index and EF did not differ significantly between groups (all p>0.05). Conclusion: The RV volume and EF of the PV annular extension group did not differ from that of the PV annular extension group. Thus, PV annular preservation technique did not show the surgical advantage compared to PV annular extension technique in this study.