• Journal of Chest Surgery
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• v.20 no.2
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• pp.404-410
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• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Korean Journal of Radiology
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• v.19 no.6
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• pp.1042-1052
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• 2018

Objective: To determine the utility of computed tomography (CT) ventricular volumes and morphometric parameters for deciding the treatment strategy in children with a hypoplastic left ventricle (LV). Materials and Methods: Ninety-four consecutive children were included in this study and divided into small LV single ventricle repair (SVR) (n = 28), small LV biventricular repair (BVR) (n = 6), disease-matched control (n = 19), and control (n = 41) groups. The CT-based indexed LV volumes, LV-to-right-ventricular (LV/RV) volume ratio, left-to-right atrioventricular valve (AVV) area ratio, left-to-right AVV diameter ratio, and LV/RV long dimension ratio were compared between groups. Proportions of preferred SVR in the small LV SVR group suggested by the parameters were evaluated. Results: Indexed LV end-systolic (ES) and end-diastolic (ED) volumes in the small LV SVR group ($6.3{\pm}4.0mL/m^2$ and $14.4{\pm}10.2mL/m^2$, respectively) were significantly smaller than those in the disease-matched control group ($16.0{\pm}4.7mL/m^2$ and $37.7{\pm}12.0mL/m^2$, respectively; p < 0.001) and the control group ($16.0{\pm}5.5mL/m^2$ and $46.3{\pm}10.8mL/m^2$, respectively; p < 0.001). These volumes were $8.3{\pm}2.4mL/m^2$ and $21.4{\pm}5.3mL/m^2$, respectively, in the small LV BVR group. ES and ED indexed LV volumes of < $7mL/m^2$ and < $17mL/m^2$, LV/RV volume ratios of < 0.22 and < 0.25, AVV area ratios of < 0.33 and < 0.24, and AVV diameter ratios of < 0.52 and < 0.46, respectively, enabled the differentiation of a subset of patients in the small LV SVR group from those in the two control groups. One patient in the small LV biventricular group died after BVR, indicating that this patient might not have been a good candidate based on the suggested cut-off values. Conclusion: CT-based ventricular volumes and morphometric parameters can suggest cut-off values for SVR in children with a hypoplastic LV.

• Journal of Chest Surgery
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• v.36 no.9
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• pp.683-686
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• 2003

Isolated right ventricular hypoplasia is a rare clinical entity. We describe a case of right ventricular hypoplasia, single atrium and spongy myocardium of left ventricle. The volume of right ventricle was half the volume of left ventricle and z-value of tricuspid valve was -4 preoperatively The patient, 6-year-old boy, underwent atrial partitioning with 3 mm fenestration, Postoperative course was smooth and he tolerated the biventricular state well during follow-up. Follow-up catherterization was done 27 months later The tricuspid valve grew well (z-value= -0.4) and atrial septal fenestration is closed spontaneously. This article reports a case of successful biventricular repair in a patient with isolated right ventricular hypoplasia.

• 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 Chest Surgery
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• v.15 no.1
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• pp.61-66
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• 1982

The present study was performed to evaluate hemodynamic effects on the pericardial patch graft for stenosis of right ventricle outflow tract in 19 patients of tetralogy of Fallot. The stenosis of right ventricle outflow tract was associated with or without pulmonary annular nar-rowing, pulmonary valvular stenosis, and hypoplastic narrowing of pulmonary artery. Total correction of tetralogy of Fallot was performed under cardiopulmonary bypass with moderate hypothermia and cardioplegic cardiac arrest. Ventricular septal defects were closed with Teflon patch graft. The chamber pressures in the heart were measured before and after a total correction of tetralogy of Fallot. The data of pressure measurement and the results of postoperative observation of pericardial patch were as followings: 1. Systolic and diastolic pressure of right ventricle was decreased after operation from $96.0{\pm}14.7/10.0{\pm}14.4mmHg$ to $61.0{\pm}13.1/8.0{\pm}9.3mmHg$. 2. Systolic and diastolic pressure of pulmonary artery was increased after operation from $18.0{\pm}5.6/10.0{\pm}5.5mmHg$ to $31.0{\pm}10.7/14.0{\pm}4.9mmHg$. 3. Preoperative pressure gradient between right ventricle and pulmonary artery was decreased immediately after operation from 78.0mmHg to 30.0mmHg. 4. It was observed that excellent widening effects of right ventricle outflow tract was resulted from pericardial patch graft. 5. No postoperative bleeding from pericardial patch graft was observed. 6. Aneurysm formation of pericardial patch was not be observed during 1 to 6 years postoperative periods.

• Journal of Chest Surgery
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• v.26 no.9
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• pp.701-704
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• 1993

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricle. Our approach was to perform complete repair at one stage. The patient who underwent single-stage repair was discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition.

• 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.21 no.5
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• pp.828-841
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• 1988

To predict the postoperative hemodynamic status of right ventricle preoperatively, a retrospective analysis was undertaken to determine the influence of pulmonary artery size on postoperative right ventricular pressure in 32 consecutive patients with tetralogy of Fallot who underwent total correction between July, 1987 to June, 1988 at the Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital. We have related the ratio of the postrepair peak systolic pressure in the right ventricle and the systemic systolic arterial pressure[PRV/Ao] to the preoperative cineangiographic measurement of pulmonary arterial tree, expressed as pulmonary artery index[PAI], the ratio of diameter of the right pulmonary artery to diameter of ascending aorta[r.PA/A.Ao], the ratio of right and left pulmonary artery to diameter of descending aorta[r.I.PA/D.Ao] There was tendency that the postrepair PRV/Ao seems to be related to the preoperative diameter of right and left pulmonary artery, but there were no statistically significant correlation with PAI, r.PA/A.Ao, r.l.PA/D. Ao to the ratio of the postoperative peak systolic right ventricular pressure and systemic systolic arterial pressure[PRV/Ao]. There was tendency to decrease the postoperative right ventricular pressure[PRV/Ao] about 11.2%[P < 0.025] within several hours than immediately after repair, but after then, there was no change of right ventricular pressure[PRV/Ao] significantly. There was good correlation of pressure change between the immediate and late postrepair right ventricular pressure[48 hour], and the derived linear regression line was; y=0.68534 0.1994[r=0.57294, P < 0.001]. There was no operative death due to residual high right ventricular pressure[PRV/Ao >0.75] related to hypoplastic pulmonary arterial development, thus we expect, for symptomatic patients even infants, that complete repair can be attempted when the pulmonary artery index[PAI] is over 108mm2/BSA, RPA/AAo is over 0.35, RPA LPA/D. Ao is over 1.36.

• Journal of Chest Surgery
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• v.24 no.3
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• pp.310-321
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• 1991

Between April 1986 and September 1990, 34 patients with a single or dominant right ventricle underwent modified Fontan procedure for definite palliation in Seoul National University Children`s Hospital. Their age at operation ranged from 8 months to 14 years [Mean 5.5 years]. The ventricular chamber was solitary and of indeterminate trabecular pattern in 6 patients. 28 patients had posteriorly located rudimentary chamber, all of which were trabecular pouches having no communication with outlet septum. The patterns of atrioventricular connection were common inlet[9], double inlet [11], left atrioventricular valve atresia [12] and right atrioventricular valve atresia with L-loop [2]. Pulmonary outflow tracts were atretic in 7 patients and stenotic in 26 patients. Major associated anomalies included anomalous systemic venous drainage [15], dextrocardia [12] and total anomalous pulmonary venous connection[3]. Shunt operations were previously performed in 13 patients and pulmonary artery banding and atrial septectomy in 1 patients. Surgery included intraatrial baffling in 26 patients, bidirectional cavopulmonary shunt in 13 patients, atrioventricular valve obliteration in 3 patients and atrioventricular valve replacement in 3 patients. Central venous pressure measured postoperatively at intensive care unit ranged from 18cm H2O to 28cm H2O [mean 23.2cm H2O]. Hospital mortality was 35.3% [12/34], all died out of low output syndrome. Suspected causes of low output syndrome include ventricular dysfunction [8], hypoplastic or tortuous pulmonary artery [2] and elevated pulmonary vascular resistance [2]. 19 patients had 31 major complications including low output syndrome [18], arrhythmia [4], acute renal failure [3] and respiratory failure [3]. Mortality rate was significantly higher in the groups receiving intraatrial baffling and AV valve replacement respectively [p<0.05]. 20 patients were followed up postoperatively with the mean follow-up period 15.0$\pm$11.6 months. There were no late death and follow-up catheterization was performed in 10 patients. Mean right atrial pressure was 15.4$\pm$6.8mmHg and ventricular contraction was reasonable in all but one case. Thus, Fontan principle can be applied successfully to all the patients with complex cardiac anomaly of single ventricle variety and better results can be anticipated with judicious selection of patient and improvement of postoperative care.

• Journal of Chest Surgery
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• v.22 no.2
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.