• Journal of Chest Surgery
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• 제54권5호
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• pp.389-392
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• 2021

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

• Korean Journal of Radiology
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• 제22권11호
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• pp.1894-1908
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• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• 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.

• Journal of Chest Surgery
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• 제33권4호
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• pp.316-319
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• 2000

Double-outlet left ventricle with ventricular septal defect and pulmonary stenosis was conventionally repaired with extracardiac conduit or pulmonary artery translocation. Here, we report an anatomically repaired double-outlet left ventricle without extracardiac conduit or pulmonary artery translocation in an 11 month old patient who had undergone palliative systemic-pulmonary shunt at a nonatal period. The location of ventricular septal defect, both great arteries and coronary arteries made it possible to reconstruct the right ventricular outflow tract using on-lay patch after incision and undercutting the tissue between the ventriculotomy and the pulmonary arteriotomy.

• 한국임상수의학회지
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• 제27권6호
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• pp.735-739
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• 2010

8개월령 말티스가 호흡곤란, 운동불내성의 증상으로 내원하였다. 흉부방사선상 환자는 심한심종대와 주폐동맥의 확장 소견을 보였고, 심초음파상 대동맥근이 우심실을 향하고 좌-우 단락을 가지고 있는 대동맥하 심실중격결손증이 확인되었다. 또한 중격결손에 의한 좌-우 단락성 혈류에 의해 폐혈관계에 과순환 소견과 폐성 고혈압에 의한 심한 폐동맥 역류가 (최고속도 4.7 m/s, 압력구배 ~88 mmHg) 확인되었다. 상기의 소견을 토대로 환자는 양대혈관 우심실기시로 진단되었다. 환자는 우심실의 혈량과부하를 줄이기 위한 이뇨제 처치(furosemide 1 mg/kg), 심장재구성을 늦추어주기 위해 spironolatcone (1 mg/kg) 및 enalapril (0.5 mg/kg) 처치와 폐의 과순환과 폐성 고혈압을 완화시켜주기 위해 sildenafil (1 mg/kg)를 처방하였다. 일주일뒤 재검에서 환자의 임상 증상은 크게 개선되었다. 현재 환자는 생존해 있고 정기적으로 모니터하고 있다.

• Journal of Chest Surgery
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• 제47권1호
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• pp.32-34
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• 2014

Anomalous aortic origin of the left subclavian artery (LSCA) from the left pulmonary artery (LPA) is a rare congenital cardiac malformation. We describe a case of LSCA from the LPA via ductus arteriosus in association with a double-outlet right ventricle, which never has been reported previously in Korea.

• Journal of Chest Surgery
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• 제23권6호
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• pp.1118-1127
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• 1990

Arterial switch operation for repair of nineteen cases of transposition of the great arteries associated with ventricular septal defect and three cases with double outlet right ventricle with subpulmonary ventricular septal defect[Taussig-Bing type DORV] was performed from November 1987 to September 1990 at the Seoul National University Children`s Hospital. Sixteen of them were under six months of age, and three were under one year of age with body weight ranged from three to fourteen kilograms. Preoperative cardiac catheterization was done in eighteen patients, in which the pressure of the left ventricle was greater than 70% of the right ventricle in all but one. Patent ductus was associated in thirteen cases[68.4%] of TGA+VSD, and atrial septal defect or patent oval foramen was in sixteen cases. Four atrial septostomy, one modified Blalock- Taussig shunt, one pulmonary artery banding, one coarctoplasty using subclavian arterial flap, were perfomed before arterial switch operation. There were five hospital deaths, all in the. patients with transposition of the great arteries with ventricular septal defect[overall mortality rate 22.7%]. Lecompte Maneuver was used in all patients, and in all patient the U-shaped flap of coronary arteries were transposed to V-shaped cleavage created in the neoaorta. Arterial defect in the neopulmonary artery was covered with 0.0625% Glutaraldehyde fixed autogenous pericardium There have been no late deaths, Postoperative cardiac catheterization and angiocardiogram in four patients has revealed no stenosis in the neopulmonary artery or neoaorta with reasonable P[RV/LV], Anatomic correction for transposition and double outlet right ventricle with subpulmonary ventricular septal defect would seem to be a good operative alternative to intraatrial switch procedures, with the advantage of incorporating the left ventricle to systemic circulation.

• Journal of Chest Surgery
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• 제23권6호
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• pp.1174-1179
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• 1990

Forty-five patients with double-outlet right ventricle[DORV] underwent complete intracardiac repair between July, 1983 and June, 1989. Patients with complete atrioventricular canal, atrioventricular discordance and uni-ventricular heart were excluded. The 32 male and 13 female patients ranged in age from 3 months to 15 years[mean 4 years]. Thirty-two patients had pulmonary stenosis. The early mortality was 11.ltd[5 /45] None of 27 died after a completely intraventricular repair. The mortality was 20%[1/5] for repair using transannular patch, 20% [1/5] for REV operation, 33.3%[1/3] for repair including extracardiac valved conduit, and 50% [1/2] for Jatene operation, respectively. Two modified Fontan procedures were performed without mortality. One died after Senning operation. Causes of early deaths included high residual right ventricular pressure[one patient] small left atrial and left ventricular volume[one patient], persisting severe pulmonary hypertension [one patient] and low cardiac output of unknown cause [two patients]. Complete heart block developed in one patient. Two late deaths occurred among the 40 operative survivors [5.0Po] from persisting severe pulmonary hypertension and bleeding at reoperation. Our results indicate that significant defects can be repaired with low mortality and morbidity.

• Journal of Chest Surgery
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• 제12권3호
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• pp.274-280
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• 1979

A case of successful surgical correction of double-outlet right ventricle with situs inversus, dextrocardia, subaortic ventricular septal defect, and pulmonic stenosis is described. The great vessels were normally related but in mirror-image arrangement. Another coronary artery anomaly, single origin of coronary artery and a large anomalous coronary artery coursed across the right ventricular outflow tract, was accompanied. Intracardiac repair with a Dacron tunnel conduit and extracardiac jumping graft with a valved conduit gave an excellent result.

• Journal of Chest Surgery
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• 제10권1호
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• pp.148-155
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• 1977

The clinical findings with cardioangiography and successful surgical treatment of a 10 year old girl with double-outlet right ventricle is reported at The Dept. of Thoracic and Cardiovascular Surgery, Korea University Hospital, College of Medicine. The patient has been suffered from intermittent cyanosis, palpitation, and exertional dyspnea since 1 year after NFSD, and a holosystolic ejection murmur of grade 4 at the left 3rd intercostal space with mild cyanosis of the lips was the only physical findings at the time of this admission. Cardiac catheterization revealed ventricular septal defect with left to right shunt of 43% and right to left shunt of 10.2%. On cardioangiography from the left ventricle revealed all of the left ventricular outflow shunted into the right ventricle through the large ventricular septal defect, and the aorta originated from the infundibular chamber of the right ventricle with left, anterior sided pulmonary artery. The atria, viscera, and ventricles were normally located, and right ventricular out-flow was narrowed with infundibular hypertrophy and pulmonary valvular stenosis. Surgical correction was accomplished by closure of the ventricular septal defect in such a way that left ventricular outflow was routed via a Teflon felt prosthetic tunnel to the aorta, and pulmonary valvulotomy with infundibulectomy Was done to pass Hegar`s dilator No. 15 for reconstruction of the right ventricular outflow tract. The patient tolerated complete repair and has continued to improve over a period of three months after operation with normal school life. Details of the disease and method of repair are presented with related references.