• 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.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.18-29
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• 1983

Thirty-two patients with a cyanotic cardiac malformations having more complex intracardiac defects than ones in a tetralogy of Fallot underwent complete intracardiac repairs in a-full-year period from July 1981 to June 1982. Twenty-two patients [68.8%] died within 30 days after surgery: Transposition of the great arteries, seven of 10 patients; Double-outlet right ventricle, four of 6 patients; Tricuspid atresia, four of 6 patients; Single ventricle, all of 4 patients; Pulmonary atresia, two of 3 patients; Double-outlet left ventricle, none of 2 patients; and Truncus arteriosus, one of a single patient. All deaths occurred with a low cardiac output syndrome or a failed off-bypass, and they were almost always accompanied with other grave postoperative complications. The complex intracardiac anatomy itself was one of the risk factors by making a complete intracardiac repair of the defects difficult in a small heart. The reconstruction of the right ventricular outflow carried a difficulty in balancing an adequate relief of the obstruction with an avoidance of making too much pulmonary valvular insufficiency as well. On the other hand, the presence of an elevated pulmonary arterial pressure and a high pulmonary vascular resistance was also the factors affecting the postoperative surviv als. The importance of detailed knowledge of intracardiac anatomy and hemodynamics from the careful preoperative evaluation of the patient was discussed along with the necessity of technical refinement of the correction.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

• Journal of Chest Surgery
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• v.49 no.3
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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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• v.20 no.4
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• pp.798-802
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• 1987

Origin of both great vessels from morphological left ventricle [DOLV] is a rare cardiac anomaly which embryologic possibility has been explained by differential conal development concept and differential canal absorption concept. Recently we had surgical experience of DOLV in 4 month-age infant weighing 5.7Kg. The chief complaints on admission were cyanosis and anoxic spell during severe crying, and right heart catheterization and right ventriculogram were performed but incorrect diagnosis was made. The operative procedures were ligation of patent ductus arteriosus, patch closure of subaortic VSD aligning aorta and pulmonary artery with left ventricle, suture closure of proximal pulmonary artery and valve and the use of extracardiac valved conduit [Carpentier-Edward l4mm] from right ventricle to distal pulmonary artery. Postoperative course was uneventful and discharged in the good condition.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.784-787
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• 1995

A 3-year old female who underwent modified Fontan operation for the double outlet right ventricle with hypoplastic left ventricle at the age of 15 month was admitted with hemoptysis, which was developed 4 days prior to visit. Cardiac catheterization revealed that multiple collaterals from descending thoracic aorta supplied the right lung and drained to the right pulmonary artery. Chest magnetic resonance imaging [MRI showed that the right lung was consolidated by the secondary long-term pulmonary congestion. We decided to perform pneumonectomy because the consolidated right lung and the back-flow from the right pulmonary aretry would worsen the present hemodynamic state of patient. Post-operative course was uneventful, and she could be discharged with good general conditions.

• Journal of The Korean Dental Society of Anesthesiology
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• v.12 no.2
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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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• v.38 no.3 s.248
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• pp.181-190
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• 2005

The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. Material and Method: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1$\~$44) and 86 patients ($68.3\%$) were male. We classified and studied this disease by the type of VSD. Result: The locations of VSD were subaortic in 79 ($62.7\%$), subpulmonary in 17 ($13.5\%$), doubly committed in 16 ($12.7\%$) and noncommitted in 14 ($11.1\%$). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0$\pm$45.1 months. The methods of total correction were intraventricular baffling in 37 ($29.4\%$), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 ($38.9\%$), intraventricular baffling with Rastelli procedure in 15 ($11.9\%$), arterial switch operation in 8 ($6.3\%$) and REV procedure in 4 ($3.2\%$), etc. Hospital mortality rate was $10.3\%$ (13 patients) and 25 reoperations were performed in 24 patients ($19.0\%$). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9$\pm$70.7 months. The percent survival and survival for freedom from reoperation at 15 years were $82.5\%$ and $66.7\%$, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. Conclusion: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

• Journal of Chest Surgery
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• v.26 no.1
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• pp.36-43
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• 1993

Sixty four children [aged 2 days to 9 years] , 58 with complete transposition of the great arteries, 5 with Taussig-Bing double outlet right ventricle, and 1 with double outlet left ventricle plus left ventricular type single ventricle, have undergone anatomic correction from November 1987 to August 1992. Eleven underwent previous operations: pulmonary artery banding[7], modified Blalock-Taussig shunt[2], coarctoplasty[2], aortic arch reconstruction[1] . Of 58 patients with TGA, Type A coronary arteries of Yacoub were seen in 50[86%]. U-shaped coroanry arterial flaps were transfered to the neoaorta using trap door technique, and neopulmonary arterial tract was constructed using glutaraldehyde fixed autopericardium with Lecompte maneuver. There were 18 hospital deaths [28.1%] with no late mortality. Mean follow-up of 20.4\ulcorner11.9 months were achieved in all survivors. Postoperative cardiac catheterizations were done in 14 cases. Mean pressure gradients of pulmonary and aortic outflow tract were 15.0 $\pm$2.6 and 4.2$\pm$1.4mmHg, mild aortic valve insufficiencies were found in 2, and mean cardiac index was 5.18$\pm$0.19 L/min/M2. We conclude that we should continue anatomic correction for the complex congenital heart anomalies with the malposition of the great arteries because myocardial function seems to be well preserved, though we are still on the learning curve.

• Journal of Chest Surgery
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• v.24 no.1
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• pp.15-25
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• 1991

From February 1988 to December 1990, 42 patients underwent so called REV operation for pulmonary stenosis or atresia with or without anomalies of ventriculoarterial connection and truncus arteriosus. The principles of operative technique are mobilization of pulmonary arterial tree beyond the pericardial reflection, transection of pulmonary trunk between the pulmonary ventricle and pulmonary artery, suture of distal pulmonary arterial stump to the upper margin of Pulmonary ventriculotomy site with absorbable suture, and anterior patch with 0.625% glutaraldehyde fixed autologous pericardium with monocusp inside it. Age at operation ranged 3-156months [mean 41.8 month] with twelve of whom infants. Operative indications were pulmonary atresia, with ventricular septal defect[16], and pulmonary stenosis with double outlet right ventricle[8], with ventricular septal defect[16], with double outlet right ventricle[8], with complete transposition of the great arteries[8], with corrected transposition of the great arteries[6], with Fallot`s tetralogy[3], and truncus arteriosus[1]. There were six hospital deaths[14%] and no late death. Twenty-four of 36 survivals were followed up more than 12 months with good clinical results. Postoperative angiocardiogram was performed in fifteen patients. Hemodynamically, two patents had residual pressure gradients along the pulmonary outflow tract, one patient showed severe pulmonary regurgitation; morphologically, there were six significant stenosis of left pulmonary arterial tree, two of whom showed significant pressure gradients. Our present experience with REV operation suggests that this technique make it possible to perform anatomic repair in a wide variety of congenital anomalies of abnormal ventriculoarterial connection associated with pulmonary outflow tract obstruction without using the prosthetic material, even in infants, with relatively low mortality and morbidity.