• Journal of Chest Surgery
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• v.30 no.12
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• pp.1242-1246
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• 1997

Criss-cross heart which is a cardiac malformation caused by abnormal rotation of the ventricles early in embryonic development, is rare but a double outlet of right ventricle in priss-cross heart is very rare. We experienced a case of criss-cross heart which is situs solidus, concordant atrioventricular connection and double outlet of rig t ventricle with remote ventricular septal defect of perimembranous inlet type. A 4-years old female was diagnosed as a double outlet of right ventricle in criss-cross heart after echocardiography, cardiac catheterization and cardiac angiography. The surgical correction was a intraventricular reconstruction of left ventricular outflow with 314 circle of 20 mm Hemashield vascular graft from the ventricular septal defect to the aorta. The patient had a temporary atrioventricular block but was recovered uneventfully, and a postoperative echocardiogram showed no left ventricular outflow obstruction, no intracardiac shunt.

• Korean Journal of Radiology
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• v.22 no.11
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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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• 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.10 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.199-207
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• 2002

Purpose : Since the successful application of total atrio-pulmonary connection(TAPC) to patients with various types of physiologic single ventricles in 1971, post-operative survival rates have reached more than 90%. However some patients have been shown to present with late complications such as right atrial thrombosis, atrial fibrillation and protein losing enteropathy eventually leading to re-operation to control the long-term complications. The aim of this study is to review the results of total cavo-pulmonary connection(TCPC) in cases with late complications after TAPC. Methods : Between Jan. 1995 and Dec. 2000, 6 patients(5 males and 1 female) underwent cardiac catheterization $11{\pm}3$ months after conversion of previous TAPC to TCPC. We compared the hemodynamic and morphologic parameters before and after TCPC and also assessed the clinical outcomes. The indications for TAPC were tricuspid atresia in 4 cases and complex double-outlet right ventricle with single ventricle physiology in 2 cases. Results : There was no peri-operative mortality and all patients were clinically and hemodynamically improved at a mean follow-up of 11 months(range : 4 to 13). However, protein losing enteropathy recurred in 2 patients; this was were successfully treated with subcutaneous administration of heparin. Right atrial pressure before TCPC was $18.0{\pm}3.6mmHg$, but baffle pressure, corresponding to right atrial pressure decreased to $14.8{\pm}3.6mmHg$ after TCPC. The size of the pulmonary arteries did not regress after TCPC. Conclusion : The conversion of TAPC to TCPC improves clinical and hemodynamic status by decreasing the right atrial pressure and by providing a laminar cavo-pulmonary flow which enhances the effective pulmonary circulation in the so-called Fontan circulation.

• Journal of Chest Surgery
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• v.31 no.12
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• pp.1183-1194
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• 1998

Background: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery(OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. Material and Method: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease(CHD) and 468 cases were acquired heart disease(AHD). The age distribution was 9 days(4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. Result: The CHD cases consisted of 940 ventricular septal defects(61.4%), 324 atrial septal defects(21.1%), 112 tetralogy of Fallot(7.3%), 46 pulmonary stenosis(3%), 38 endocardial cushion defects(2.5%), 15 valsalva sinus ruptures(1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles(0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements(36 aortic valve replacements(AVR), 188 mitral valve replacements(MVR), and 2 tricuspid valve replacements(TVR), among these were 71 cases of double valve replacements(AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty(TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery(CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. Conclusion: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6%(72/2,000).

• Journal of Chest Surgery
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• v.32 no.9
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• pp.781-789
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• 1999

Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.