• Title/Summary/Keyword: Congenital

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Clinical Features of Cricopharyngeal Incoordination in Newborns and Infants (신생아 및 영아기 윤상인두 협조불능의 임상적 고찰)

  • Han, Young-Mi;Park, Jae-Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.116-121
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    • 2008
  • Purpose: Cricopharyngeal incoordination is a rare cause of swallowing difficulties in newborns and infants; it is characterized by delayed pharyngeal contractions related to cricopharyngeal relaxation. Dysphagia and repeated aspiration are common findings despite normal sucking. We conducted this study to assess the clinical features of cricopharyngeal incoordination in newborns and infants. Methods: An analysis of the clinical data from 17 patients with cricopharyngeal incoordination who were admitted to the Department of Pediatrics, Pusan National University Hospital, between 2000 and 2006 was conducted retrospectively. The diagnosis of cricopharyngeal incoordination was established by the clinical characteristics and the videofluoroscopic swallowing studies. Results: The male to female ratio was 1:1.1 (males 8, females 9) the age range 1 to 60 days. The body weight of 11 patients (64.7%) was less than the $10^{th}$ percentile at diagnosis. Six patients (35.3%) were born prematurely. The associated anomalies or diseases were chromosomal anomaly (2 cases), congenital heart disease (3 cases), and laryngomalacia, hypoxic brain damage or neonatal seizures (1 case each). The chief complaints of patients were recurrent aspiration pneumonia (10 cases), feeding difficulty (9 cases), dyspnea (4 cases), and chocking (4 cases). The severity of aspiration on the videoesophagogram or esophagogram was mild in 12 cases. The correlation between the severity of aspiration and the duration of tube feeding after the diagnosis was significant (p<0.05). Conclusion: Cricopharyngeal incoordination should be considered in the differential diagnosis of newborns and infants, without known risk factors associated with swallowing dysfunction, when they present with unexplained respiratory problems. Although the prognosis of cricopharyngeal incoordination is good, early diagnosis and tube feeding are recommended to prevent the complications associated with this disorder.

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Colonization Rate and Control of Vancomycin-Resistant Enterococci in the Neonatal Intensive Care Unit (일개 신생아중환자실 반코마이신 저항 장구균(VRE)의 유행 양상과 조절)

  • Seo, Jung Ho;Nam, Ga Yeon;Park, Kyung Hee;Byun, Shin Yun;Park, Su Eun
    • Pediatric Infection and Vaccine
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    • v.17 no.1
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    • pp.1-8
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    • 2010
  • Purpose : Recently, vancomycin-resistant enterococci (VRE) have become one of the major nosocomial pathogens in Korea. However, there have been few studies on the epidemiology of VRE colonization among neonates. In this study, we investigated the prevalence of VRE colonization, risk factors for VRE, and how to control the spread of VRE infection in the Neonatal Intensive Care Unit (NICU) of Pusan National University Hospital (PNUH). Methods : We retrospectively reviewed medical records of 192 neonates who were admitted to the NICU of PNUH from March 2006 to March 2007. Surveillance cultures from rectal swabs for detecting VRE were obtained weekly during the study period. We analyzed the prevalence of VRE and various risk factors. Results : The rate of VRE colonization among NICU patients was 25% (48/192). Thirty five of these VRE colonized patients were transferred to the NICU from other local hospitals. Compared with the non-VRE group, the risk factors associated with VRE colonization were lower birth weight, congenital heart disease, applied mechanical ventilation, use of a central venous catheter, chest tubing, a history of surgery, and use of antibiotics. Conclusion : VRE colonization among patients admitted to the NICU is rapidly increasing. Monitoring and managing premature neonates from the beginning of the birth process, avoiding many invasive procedures, avoiding antibiotics such as vancomycin and third generation cephalosporin are important for preventing the emergence and spread of VRE colonization in the NICU.

Valve Replacement in Children (소아심장판막치환술)

  • 김재현;이광숙;윤경찬;유영선;박창권;최세영
    • Journal of Chest Surgery
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    • v.32 no.4
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    • pp.341-346
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    • 1999
  • Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

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Pulmonary Valve Replacement with Tissue Valves After Pulmonary Outflow Tract Repair in Children (소아에서 폐동맥유출로 재건 후 시행한 조직판막을 이용한 폐동맥판 대치술)

  • Lee, Jeong-Ryul;Hwang, Ho-Young;Chang, Ji-Min;Lee, Cheul;Choi, Jae-Sung;Kim, Yong-Jin;Rho, Joon-Ryang;Bae, Eun-Jung
    • Journal of Chest Surgery
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    • v.35 no.5
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    • pp.350-355
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    • 2002
  • Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

Twenty-one Year Experience with Right Ventricle to Pulmonary Artery Conduit Interposition (우심실-폐동맥 간 도관 이식술의 21년간의 경험)

  • Kwak, Jae-Gun;Yoo, Jae-Suk;Kim, Yong-Jin;Kim, Woong-Han;Lee, Jeong-Ryul
    • Journal of Chest Surgery
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    • v.41 no.4
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    • pp.417-422
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    • 2008
  • Background: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. Material and Method: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was $3.2{\pm}4.9$ years (range: 7 days$\sim$45 years) and the mean body weight was $12.5{\pm}8.7\;kg$ (range: $2.4\sim76.3\;kg$). Result: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was $6.3{\pm}5.2$ years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. Conclusion: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV outflow.

Minimal Invasive Surgery: A National Survey of Its Members by the Korean Association of Pediatric Surgeons (최소 침습 수술: 대한소아외과학회 회원을 대상으로 한 전국조사)

  • Kim, Dae Yeon;Kim, I.S.;Kim, H.Y.;Nam, S.H.;Park, K.W.;Park, W.H.;Park, Y.J.;Park, J.H.;Park, J.Y.;Park, J.S.;Park, J.Y.;Boo, Y.J.;Seo, J.M.;Seol, J.Y.;Oh, J.T.;Lee, N.H.;Lee, M.D.;Jang, J.H.;Jung, K.H.;Jung, S.Y.;Jung, S.E.;Jung, S.M.;Jung, E.Y.;Jung, J.H.;Cho, M.J.;Choi, K.J.;Choi, S.J.N.;Choi, S.O.;Choi, S.H.;Choi, Y.M.;Hong, J.
    • Advances in pediatric surgery
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    • v.20 no.1
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    • pp.1-6
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    • 2014
  • Minimal invasive surgery (MIS) has rapidly gained acceptance for the management of a wide variety of pediatric diseases. A questionnaire was sent to all members of the Korean Association of Pediatric Surgeons. Thirty one members (25.4%) took part in the survey that included data for the year 2012: demographic details, opinion regarding minimal invasive surgery and robotic surgery, spectrum of minimally invasive operations, and quantity of procedures. 48.4% of the respondents had more than 10 years experience, 35.5% less than 10 years experience, and 16.1 % had no experience. The respondents of the recommend MIS and perform MIS for surgical procedures are as follow; inguinal hernia (61.3%), simple appendicitis (87.1%), complicated appendicitis (80.6%), reduction of intussusceptions (83.9%), pyloromyotomy (90.3%), fundoplication (96.8%), biopsy and corrective surgery of Hirschsprung's disease (93.5%/90.3%), imperforate anus (77.4%), congenital diaphragmatic hernia (80.6%), and esophageal atresia (74.2%). The MIS procedures with more than 70% were lung resection (100%), cholecystectomy (100%), appendectomy (96.2%), ovarian torsion (86.7%), fundoplication (86.8%), hiatal hernia repair (82.6%), and splenectomy (71.4%). The MIS procedures with less than 30% were congenial diaphragmatic hernia reapir (29.6%), esophageal atresia (26.2%), correction of malroatation (24.4%), inguinal hernia repair (11.4%), anorectal malformation (6.8%), Kasai operation (3.6%).

Effects of Single Fetal Death on Mother and Live Co-twin in Twin Pregnancy (쌍태 임신에서 일측 태아의 자궁내 사망이 산모와 생존아에 미치는 영향)

  • Kim, So Youn;Chung, Hae Yul;Back, Hee Jo;Choi, Ic Sun;Cho, Chang Yee;Choi, Young Youn
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1512-1518
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    • 2002
  • Purpose : Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. Methods : A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. Results : Gestational age was $33.7{\pm}3.2weeks$, birth weight was $1,992{\pm}592g$. Interval between one fetal death being detected and the delivery of a live co-twin was $32.4{\pm}29.5days$. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. Conclusion : We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.

A Clinical Study of Changes in Serum Electrolyte Concentration During and After Extracorporeal Circulation with Heart-Lung Machine (심폐기 체외순환에 의한 혈청 전해질 변동에 관한 연구)

  • 김근호
    • Journal of Chest Surgery
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    • v.11 no.4
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    • pp.404-415
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    • 1978
  • The present study was carried out to develop the better measures for safety of open heart surgery under extracorporeal circulation (ECC) with Heart-Lung-Machine by preventing changes in the concentrations of serum electrolytes during and after ECC. For this purpose, the cocentrations of serum electrolytes were measured before, during, and after ECC in 21 patients with congenital and acquired heart diseases who received open heart surger, - under ECC using Heart-Lung-Machine. Also considered was the development of safety measured by which changes in serum electrolyte concentrations were prevented during and after open heart surgery under ECC. The mean values for serum sodium levels were observed to be ; $13.14{\pm}0.47$mEq./L. for the samples obtained before ECC. $139.59{\pm}0.68$mEq./L. for the samples obtained 10 minutes after ECC and $138.0{\pm}0.68$mEq./L. for the samples obt"ined 24 hours after ECC. These results indicate that serum sodium concentrations were \\'ithin normal range during and until 24 hours after ECC. 2) The concentrations of serum chloride were found to be $105.38{\pm}0.70$105.38$\pm$0. 70 mEq./L. for the samples collected before ECC, $105.07{\pm}1.01$mEq./L. for the Simples collected 24 minutes aiter ECC and $101.95{\pm}1.09$mEq./L. for the samples collectect 24 hours afte ECC. As was tile case with serum sodium levels, no significant changes were observed in serum chloride levels during and 24 hours after ECC. 3)With proper provisions of potassium chloride solution during ECC, the concentrations of serum potassium were found to be $4.22{\pm}0.06$mEq./L. for the samples removed before EeC, $4.06{\pm}0.14$mEq./L. for the samples removed 10 minutes after ECC and $4.39{\pm}0.07$ mEq./L. for the samples removed 24 hours after ECC. 4)The concentrations of serum calcium were also maintained within normal during and after ECC; $9.15{\pm}0.14$mg/dl for the serum collected before ECC, $8.36{\pm}0.21$mg/dI for the serum collected 10 minutes after ECC and $8.47{\pm}0.14$mg/dl 21 hours after ECC. The maintenance of serum calcium level within normal throughout ECC was achieved by parenteral administrations of calcium gluconate as frequent as required. 5) As were the cases with serum potassium and calcium, the concentrations of plasma bicarbonate was regulated within normal range during and after ECC, only when sodium bicarbonate solution was admini"tered parenterally as it was required; $23.7{\pm}0.50$mEq./L. for the serum collected before ECC. $22.33{\pm}1.09$mEq.lL. for the serum collected 10 minutes after ECC and $25.3{\pm}0.96$mEq./L. for the serum collected 24 hours after ECC. The above results indicate tha t during and after ECC serum sodium and chloride levels remined unchanged without any provision of normal saline, while serum potassium, calcium, and bicarbonate concentrations were kept within normal limits only when these ealectrolytes were administered through parenteral routes. With these results it can be concluded that serum potassium, calcium, and bicarbonate levels should be determined as often as possible during and after ECC and that in order to maintain serum electrolyte levels within normal these electrolytes in the forms of potassium chloride, calcium gluconate, and sodium bicarbonate shou'd be given parenterally as they were found to be required.

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Analysis of the Causes of and Risk Factors for Mortality in the Surgical Repair of Interrupted Aortic Arch (대동맥궁 단절증 수술 사망 원인과 위험인자 분석)

  • Kwak Jae Gun;Ban Ji Eun;Kim Woong-Han;Jin Sung Hoon;Kim Yong Jin;Rho Joon Ryang;Bae Eun Jung;Noh Chung Il;Yun Yong Soo;Lee Jeong Ryul
    • Journal of Chest Surgery
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    • v.39 no.2 s.259
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    • pp.99-105
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    • 2006
  • Background: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. Material and Method: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. Result: There were .14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. Conclusion: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.

Surgical Outcome of Biventricular Repair for Double-outlet Right Ventricle: A 18-Year Experience (양대혈관우심실기시증에 대한 양심실 교정의 수술 성적: 18년 치험)

  • 이정렬;황호영;임홍국;김용진;노준량;배은정;노정일;윤용수;안규리
    • Journal of Chest Surgery
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    • v.36 no.8
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    • pp.566-575
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    • 2003
  • We reviewed our 18-year surgical experience of biventricular repair for double-outlet right ventricle. Material and Method: One hundred twelve consecutive patients (80 males and 32 females) who underwent biventricular repair for double-outlet right ventricle between May 1986 and September 2002 were included. We assessed risk factors for early mortality and reoperation. Reoperation-free survival rate and actual survival rate were analysed. Result: Most common type of ventricular septal defect was subaortic (n=58, 52%) and non-committed type was second most common (n=32, 29%). Four different surgical methods were used: intraventricular baffle repair (n=71 , 63%): right ventricle to pulmonary ariery conduit interposition or REV with left ventricle to aorta baffle repair (n=24, 21 .4%): arierial switch operation with left ventricle to pulmonary artery baffle (n=14, 12.5%): Senning atrial switch operation with left ventricle to pulmonary artery baffle (n=3, 2.7%). Thirty four patients(30%) underwent palliative procedures before definite repair. Twenty three patients (21%) required reoperations. There were 12 (10.7%) early deaths and 4 late deaths. Age younger than 3 months at repair (p=0.003), cardiopulmonary bypass and aortic cross clamp time (p=0.015, p=0.067), type of operation (arterial switch operation) (p <0.001) and type of ventricular septal defect (subpulmonic type) (p=0.002) were revealed as risk factors for early death in univariate analysis, while age under 3 months was the only significant risk factor in multivariate analysis. Patients younger than 1 year of age (p=0.02), pulmonary artery angioplasty at definitive repair (p=0.024), type of ventricular septal defect (non-committed) (p=0.001), type of operation (right ventricle to pulmonary artery conduit interposition and REV operation) (p=0.028, p=0.017) were risk factors for reoperation in univariate analysis but there was no significant risk factor in multivariate analysis. Follow-up was available on 91 survivals with a mean duration of 110.8$\pm$56.4 (2~201) months. 5, 10 and 15 year survival rates were 86.5%, 85% and 85% and reoperation free survival were 85%, 71.5%, 70%. Conclusion: Age under 3 months at repair, subpulmonic ventricular septal defect and arterial switch operation were significant risk factors for early mortality. Patients with non-committed ventricular septal defect and who underwent conduit interposition or REV operation were risk factors for reoperation. With careful attention to chose best timing and surgical approach depending on morphologic characteristics, biventricular repair for double outlet right ventricle can be achieved with good long-term outcome.