• Journal of Chest Surgery
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• 제30권11호
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• pp.1121-1124
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• 1997

심실 중격이 건재한 대혈관전위증 환아가 동맥 전환술의 시기를 넘겨 단기간에 걸친 2단계의 동맥 전환술로 성공적인 치료를 했기에 보고하는 바이다. 4개월 여 환아는 심한 청색증과 호흡곤란, 대장의 허혈성 증상으로 보이는 잦은 설사 등으로 입원하였다. 입원당시 초음파 검사에서 심실 중격은 좌측으로 심하게 만곡되 어있는 소견을 보였고, 음도자 검사상 좌우 심실 수축기 압력은 각각 40/4 mmHg, 85/2mmHg였다. 우측 체 폐동맥 단락술과 폐동맥 교약술의 1단계 수술을 시행한 15일 후에는 좌우 심실 수축기압의 비가 105/90으로 향상되었고 그로부터 2일 후 우측 체폐동맥 단락 및 폐동맥 교약끈의 해체와 동맥 전환술을 시행하였다. 수술후 환자는 특별한 합병증없이 정상적인 심기능을 보였다.

• Korean Circulation Journal
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• 제53권6호
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• pp.406-417
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• 2023

Background and Objectives: Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. Methods: In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. Results: Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO₂] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO₂ was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. Conclusions: We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

• Korean Journal of Radiology
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• 제20권6호
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• pp.956-966
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• 2019

Objective: To characterize the changes in right ventricular (RV) volume, volume load, and function measured with cardiac computed tomography (CT) over the entire time course of tetralogy of Fallot (TOF). Materials and Methods: In 374 patients with TOF, the ventricular volume, ventricular function, and RV volume load were measured with cardiac CT preoperatively (stage 1), after palliative operation (stage 2), after total surgical repair (stage 3), or after pulmonary valve replacement (PVR) (stage 4). The CT-measured variables were compared among the four stages. After total surgical repair, the postoperative duration (POD) and the CT-measured variables were correlated with each other. In addition, the demographic and CT-measured variables in the early postoperative groups were compared with those in the late postoperative and the preoperative group. Results: Significantly different CT-based measures were found between stages 1 and 3 (indexed RV end-diastolic volume [EDV], 63.6 ± 15.2 mL/m² vs. 147.0 ± 38.5 mL/m² and indexed stroke volume (SV) difference, 7.7 ± 10.3 mL/m² vs. 32.2 ± 16.4 mL/m²; p < 0.001), and between stages 2 and 3 (indexed RV EDV, 72.4 ± 19.7 mL/m² vs. 147.0 ± 38.5 mL/m² and indexed SV difference, 5.7 ± 13.1 mL/m² vs. 32.2 ± 16.4 mL/m²; p < 0.001). After PVR, the effect of RV volume load (i.e., indexed SV difference) was reduced from 32.2 mL/m² to 1.7 mL/m². Positive (0.2 to 0.8) or negative (-0.2 to -0.4) correlations were found among the CT-based measures except between the RV ejection fraction (EF) and the RV volume load parameters. With increasing POD, an early rapid increase was followed by a slow increase and a plateau in the indexed ventricular volumes and the RV volume load parameters. Compared with the preoperative data, larger ventricular volumes and lower EFs were observed in the early postoperative period. Conclusion: Cardiac CT can be used to characterize RV volume, volume load, and function over the entire time course of TOF.

• Journal of Chest Surgery
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• 제34권11호
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• pp.854-857
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• 2001

양대혈관 좌심실기시는 양대혈관이 좌심실에서 기시하는 드문 선천성 심장기형으로 정확하게 진단하기가 어렵다. 환아는 생 후 2개월에 대동맥축착교정술 및 폐동맥교약술을 받았던 3세된 남아로 대동맥하 심실중격결손, 단일관상동맥을 동반한 양대혈관 좌심실기시로 진단되어 심실중격결손의 첩포봉합, 난원공개존의 봉합 폐동맥 교약부 절제, 그리고 폐동맥간을 좌심실에서 분리 후 우심실로 전위시켜 우심실 유출로 재건술을 받았다. 본 술식은 술 후 좋은 혈역학적 특성을 보였고, 자가조직을 사용함으로써 전위된 폐동맥의 성장잠재성과 판막 기능의 보존으로 술 후 폐동맥 폐쇄부전과 관련된 우심실기능부전의 가능성이 줄어드는 장점이 예상되므로 문헌 고찰과 함께 보고한다.

• Clinical and Experimental Pediatrics
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• 제48권11호
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• pp.1256-1256
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• 2005

심실중격결손을 동반하지 않은 폐동맥 폐쇄(pulmonary atresia with intact ventricular septum)는 전체 선천적 심장병의 1%를 차지하는 드문 질환으로 형태학적 특성에 따라 다양한 치료적 접근이 필요하다. 이에 저자들은 정상 크기발달의 우심실을 가진 심실중격결손을 동반하지 않은 폐동맥 폐쇄가 있는 신생아에서 풍선판막성형술 후 지속적인 저산소증을 보여 동맥관 스텐트를 이용하여 거의 정상적인 심장구조와 기능으로 호전된 1례를 경험하였기에 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제50권3호
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• pp.343-352
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• 2001

연구배경 : 만성 폐쇄성 폐질환 기능의 평가를 위해서 여러 가지 비침습적, 침습적 방법이 이용되나 그 정확도나 실용성에 있어서 한계가 있었다. 최근 도플러 심초음파상의 수축기, 이완기 및 박출시간을 조합한 기능지수인 Tei 지수가 우심실기능을 평가하는 유용한 방법으로 알려지고 있어 저자 등은 만성 폐쇄성 폐질환 환자의 Tei 지수를 구하여 우심실 기능을 평가하고 폐기능검사와의 상관관계를 알아보고자 하였다. 대상 및 방법 : 만성 폐쇄성 폐질환 환자 26예와 대조군 10예을 대상으로 간헐파 도플러를 이용하여 도플러 간격을 측정하였다. Tei 지수는 등용성 수축시간(ICT)과 등용성 이완시간(IRT)의 합을 박출시간(ET)으로 나누어 구하였고, PEP, ICT/ET, PEP/ET, IRT/ET를 측정하여 환자군과 대조군을 비교하였다. 폐기능과 Tei 지수의 상관관계를 구하였고, Tei 지수와 다른 도플러 간격의 상관관계를 알아보았다. 결 과 : Tei 지수는 환자군에서 대조군에 비해 유의하게 증가되어 있었고($0.45{\pm}0.17$ vs. $0.27{\pm}0.03$, p<0.01), ICT/ET ($0.32{\pm}0.08$ vs. $0.25{\pm}0.05$, p<0.05), PEP/ET ($0.46{\pm}0.10$ vs. $0.38{\pm}0.06$, p<0.05), IRT/ET ($0.29{\pm}0.16$ vs. $0.15{\pm}0.15$, p&0.05) 등은 대조군보다 증가되어 있었으며 박출시간은 단축되어있었다($255.2{\pm}32.6$ vs. $314.2{\pm}16.5$, p<0.05). 환자군에서 Tei 지수는 1초간 노력성 호기량과 역상관성을 보였고 (r=-0.469), 중증 폐기능 장애가 있는 3기 환자는 1기나 2기 환자에 비해 Tei 지수가 증가되어 있었다. 환자군에서 Tei 지수는 박출시간과 역상관성을 보였고 (r=-0.469), ICT/ET(r=0.453), PEP/ET(r=0.480), IRT(r=0.812), IRT/ET(r=0.896) 는 Tei 지수와 유의한 상관관계가 있었다. 결 론 : Tei 지수는 만성 폐쇄성 폐질환 환자에서 정상 대조군에 비해 유의하게 증가되어 있으며 폐기능 지표와도 유의한 연관성을 보여 만성 폐쇄성 폐질환 환자의 우심실 기능을 평가하는데 유용한 검사법으로 사료된다.

• 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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• 제22권5호
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• pp.753-759
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• 1989

Recently, the arterial switch operation has received more interest and increased application for many patients with TGA [Transposition of the Great Arteries]. Not all patients, however, are suitable candidates for this approach, and its success will be measured against the Senning procedure. From June 1982 through June 1989, 48 consecutive patients underwent correction of TGA by a modification of the Senning procedure in our hospital. 34 were males and 14 females, with ages ranging from 3 months to 99 months [mean age 14 months]. The patients were divided into two groups, group I [TGA with intact ventricular septum or without significant pulmonary hypertension] and group II [TGA with significant pulmonary hypertension]. The hospital mortality was 9.5 % in group I and 55.6 % in group lI. 31 patients were discharged from the hospital after a Senning operation and have been followed a mean of 31.2 months. There were 5 late deaths, 3 in group I and 2 in group II. All patients were followed up with EKG, echocardiogram, and in several cases, Holter monitor and cardiac catheterization and angiography were also performed. There have been arrhythmias in 7, superior vena caval obstruction in 5, pulmonary venous obstruction in 2 and tricuspid insufficiency and/or right ventricular dysfunction in 16 patients. The Senning operation for Simple TGA can be performed with a low operative mortality and morbidity, but tricuspid insufficiency and/or depressed right ventricular function can be a problem with prolonged follow up.

• Journal of Chest Surgery
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• 제43권1호
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• pp.63-66
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• 2010

심실중격결손증 중 심첨부 근육형은 드문 심기형이다. 특히 소아에서는 시야확보가 어려워 심방실을 통한 접근은 치료가 힘들다. 좌심실을 통한 치료는 장기적으로 좌심실 기능부전을 가져온다. 혈관 중재술을 통한 치료는 아직 만족스런 결과를 얻기 힘들며 어린 소아에서는 가능하지도 않다. 심첨부 근육형 심실중격결손증의 수술 시 우심첨부 절개술이 시야확보가 용이하며 심실기능에 손상을 주지 않고 안전하고 성공적인 교정술이 가능하였음을 보고한다.

• Journal of Chest Surgery
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• 제48권5호
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• pp.351-354
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• 2015

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.