• 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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• 제18권2호
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• pp.288-292
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• 1985

The division of the right ventricle into two chambers by aberrant muscle bands traversing the sinus portion, Double Chambered Right Ventricle[DCRV], is a relatively rare congenital cardiac malformation. Double Outlet Right Ventricle[DORV], basically recognized by the origin of both great arteries from the morphologic right ventricle, is also a rare anomaly; its frequency has been reported as approximately 0.09 case per 1,000 birth. DORV associated with DCRV, unusual combination, is even more rare; only a few known cases have been recorded previously in the literature. This report presents our surgical experience with this rare anomaly, DORV with DCRV.

• Journal of Chest Surgery
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• 제37권1호
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• pp.76-79
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• 2004

비수임형 양대혈관 우심실기시증은 심실중격결손(DORV with remote VSD)이 대동맥과 상당히 떨어져 있어 양심실 교정에 상당한 어려움이 따른다. 본 교실에서는 심실중격결손과 폐동맥을 배플(baffle)을 이용하여 연결한 후 대혈관 전환술(arterial switching operation)을 시행하여 양심실 교정을 성공적으로 치험하였기에 보고하는 바이다.

• 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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• 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.

• 대한치과마취과학회지
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• 제12권2호
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• pp.115-119
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• 2012

Double outlet of right ventricle (DORV) refers to a congenital heart disease in which pulmonary and systemic circulation originates from the right ventricle. In the patient with DORV, it is important to maintain the balance between pulmonary and systemic circulation in anesthetic management. A 4-year-old boy with DORV, who underwent a Blalock-Taussig shunt operation, was transferred to the clinic with a chief complaint of multiple caries. Due to poor cooperability, it was impossible to treat the caries without sedation or general anesthesia. We planned to sedate him with consideration with detrimental effects associated with positive pressure ventilation for dental treatment. After a prophylactic administration of antibiotics, sevoflurane was administered through T-cannula site. Throughout the treatment, His blood remained stable around 80/40 mmHg, oxygen saturation remained around 91%. After 3 hour of sedation with sevoflurane (end-tidal sevoflurane con 1-1.8 vol%), he fully regained consciousness, and discharged from hospital without complications. In case of DORV patient, deep sedation with sevoflurane may be used as effective method of behavioral management during dental treatment.

• Journal of Chest Surgery
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• 제49권3호
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• pp.190-194
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• 2016

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

• Journal of Chest Surgery
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• 제16권3호
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• pp.342-348
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• 1983

The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit was initially proposed for Tricuspid Atresia, but it and its modifications have now gained a much wider application than initially intended. From September 1978 to July 1983, Atriopulmonary anastomosis has been performed in 23 patients, 2 months to 19 years of age, Seoul National University Hospital. The diagnoses were Tricuspid Atresia [TA] in 13, Univentricular Heart [UVH] in 9 and one case of Double Outlet of Right Ventricle [DORV]. Previous procedures included two Glenn shunts and one Blalock-Taussl8 shunt. Among these 23 patients, 10 patients had Right Atrial to Pulmonary Artery conduit, with a valve in 7 and without in 3. The remaining 13 patients had direct anastomosis between RA to PA. There were 14 early deaths, 7 of 13 TA patients, 6 of 9 UVH patients and one DORV, and the total hospital mortality was 60%. But there were no later deaths, 9 of these 23 patients survived operation and are presently alive. The Fontan procedure can be done with an acceptable low mortality with good functional results for Tricuspid Atresia and other complex lesion in foreign hospital, but till now our results revealed much higher risks. For our good operative results and effective patients selection, we must clarify the exact condition of pulmonary arterial system and accumulate much more experiences and technique.

• Journal of Chest Surgery
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• 제30권7호
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• pp.641-646
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• 1997

세종병원 흉부외과에서는 1995년 5월부터 1996년 9월까지 비상관성 심실중격결손을 동반한 양대혈관우심 실기시증 환자 7례를 대상으로 하였다. 연령분포는 2세에서 9세로 평균 3.4$\pm$2.7세였으며 남녀비는 남자가 5례, 여자가 2례이었다. 술전 전례에서 심초음파 및 심도자 검사를 시행하였다. 심실중격결손은 7례모두가 비 상관성으로 막주변입구부형이었으며, 폐동맥협착이 5례, 폐동맥폐쇄가 2례 있었다. 5례의 환자에서 삼첨판막 건삭이 비정상적으로 누두부중격에 붙어있는 소견을 보여주었다. 수술은 2례에서 심실내교정술 및 폐동맥확 장술을, 3례에서 Rastelli술식을, 2례에서 REV 술식을 시행하였다. 술후 사망한 환자는 없었고, 추적조사는 1 개월에서 18개월로 평균 10$\pm$6개월 이었다. 비상관성 심실중격결'손을 가지는 양대혈관우심실기시증 환자에서 삼첨판막 건삭이 비정상적으로 누두부 중격에 붙어있는 경우에 건삭 또는 건삭을 포함한 누두부 심근의 재이식술을 이용함으로써 양심실성 교정술 이 가능하게 되었으며 이로 인해 정상적인 해부 구조와 생리를 가능하게 하는 잇점을 얻을 수 있을 것으로 사료된다. 결론적으로 추적기간이 아직 짧지만 수술결과는 비교적 만족할만하였으며, 무엇보다도 중요한 것은 수술 전에 정확한 검사 및 병태생리학적인 이해가 필요하며 그에따른 적절한 수술이 이루어져야 할 것으로 사료된다.

• Journal of Chest Surgery
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• 제12권3호
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• pp.225-232
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• 1979

A 15-year-old girl underwent successful surgical correction of double-outlet right ventricle [S.D.L.] subaortic ventricular septal defect, patent foramen ovale, and pulmonary hypoplasia with valvular stenosis. The operation consisted of an internal baffling connecting the left ventricle to the aorta through the ventricular septal defect. The pulmonary stenosis was corrected with the method of connection the right ventricle to the pulmonary artery bifurcation using the Hancock valve[18mm] contained conduit. This rare type of DORV seemed to be suitable for corrective surgery, and the patient`s condition is very good until present time (post operative 7 months).