• 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.

• Journal of Chest Surgery
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• v.23 no.2
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• pp.213-221
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• 1990

We have experienced 62 cases of modified Fontan operations in Seoul Nat’l University Hospital from Apr. 1986 to Jul. 1989, They were 38 males and 24 females, and their age was ranged from 16 months to 15.5 years of age. [mean age : 5.73$\pm$2.99 years] There were 16 operative deaths and 2 late deaths, therefore 29% of overall mortality. Their diagnoses were as follows; 28 single ventricle, 11 tricuspid atresia, 6 DORV with LV hypoplasia, 3 pulmonary atresia with hypoplastic RV, 3 TGA with hypoplastic RV, 3 cor\ulcornerGA with hypoplastic LV and PA, 6 AV canal defects with PA, and 2 others. Low cardiac output and pleural effusion were developed frequently, so we divide 40 patients into some groups to analyze the physiologic and anatomic causes of them. By the degree of the LCO, group A was no LCO[mean amount of inotropics used: 0-5 \ulcornerg/kg/min] with 17 cases, B mild LCO [5-10] with 11, C moderate to severe LCO but alive[>10] with 8, D severe LCO to death with 4 cases. For the pleural effusion, group 1 was to be removed the chest tube within 1 week with 8 cases, group II within 3 weeks with 21 cases, group III beyond 3 weeks with 12 cases. We considered their age, diagnosis, pulmonary artery size[PA index], pulmonary artery abnormality, palliative shunt, systemic ventricular type, pulmonary artery wedge pressure, as preoperative factors, and operative methods, and as postoperative factors, CVP, LAP, arrhythmia, thrombosis, atrioventricular valvular insufficiency, etc. In the view of LCO, pulmonary artery size and PCWP were statistically significant [P<0.05], and arrhythmia, A-V valve insufficiency were inclined to the group C and D Pleural effusion was influenced by the pulmonary artery size, pulmonary artery resistance, PCWP, and CVP significantly. [P<0.05] And arrhythmia, residual shunt, and A-V valvular insufficiency were inclined to group II and III, too. As a results, the followings are to be reminded as the important factors at the care of post-Fontan LCO, and persistent pleural effusion [1] pulmonary artery size, [2] pulmonary artery resistance, [3] PCWP, [4] CVP, [5] arrhythmia, [6] residual shunt, [7]A-V valvular insufficiency.

• 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.20 no.4
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• pp.751-759
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• 1987

We have experienced 4 cases of modified Fontan operation for Tricuspid atresia type III from February 1983 to May 1987 at the department of Thoracic 8 Cardiovascular Surgery, Seoul National University Hospital. There were 3 cases with left juxtaposition of right atrial auricle. The operatic mortality rate was 25% [one case death].

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.579-587
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• 2006

Background: Preoperative risk analysis for Fontan candidates is still less than optimal in that patients with apparently low risks may have poor surgical outcome; prolonged pleural drainage, protein losing enteropathy, pulmonary thromboembolism and death. We hypothesized that low pulmonary vascular compliance (PVC) is a risk factor for prolonged pleural effusion drainage after the Fontan operation. Material and Method: A retrospective review of 96 consecutive patients who underwent the Extracardiac Fontan procedures (median age: 3.9 years) was performed. Fontan risk score (FRS) was calculated from 12 categorized preoperative anatomic and physiologic variables. PVC $(mm^2/m^2{\cdot}mmHg)$ was defined as pulmonary artery index $(mm^2/m^2)$ divided by total pulmonary resistance $(W.U{\cdot}/m^2)$ and pulmonary blood flow $(L/min/m^2)$ based on the electrical circuit analogue of the pulmonary circulation. Chest tube indwelling time was log-transformed (log indwelling time, LIT) to fit normal distribution, and the relationship between preoperative predictors and LIT was analyzed by multiple linear regression. Result: Preoperative PVC, chest tube indwelling time and LIT ranged from 6 to 94.8 $mm^2/mmHg/m^2$ (median: 24.8), 3 to 268 days (median: 20 days), and 1.1 to 5.6 (mean: 2.9, standard deviation: 0.8), respectively. FRS, PVC, cardiopulmonary bypass time (CPB) and central venous pressure at postoperative 12 hours were correlated with LIT by univariable analyses. By multiple linear regression, PVC (p=0.0018) and CPB (p=0.0024) independently predicted LIT, explaining 21.7% of the variation. The regression equation was LIT=2.74-0.0158 PVC+0.00658 CPB. Conclusion: Low pulmonary vascular compliance is an important risk factor for prolonged pleural effusion drainage after the extracardiac Fontan procedure.

• Journal of Chest Surgery
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• v.34 no.1
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• pp.85-90
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• 2001

폰탄술식 후 수술사망률이 감소하고 생존자가 증가함에 따라 대정맥-폐동맥 순환의 부자연스러운 생리와 관련된 합병증이 발생하게 되었다. 이러한 혈역학적인 문제로 인하여 발생한 합병증 중의 하나가 단백소모성 장질환이다. 단백소모성 장질환은 폰탄술식 후에 발생하는 드물지만 매우 위험한 합병증이다. 많은 다른 치료경향이 제안되었지만 성공적인 치료보고는 제한되어 있다. 폰탄술식후 단백소모성 장질환이 발생한 3명의 환아에서 완전 대정맥-폐동맥 연결로 전환을 시행하였다. 완전 대정맥 폐동맥 연결로 전환함이 만족할 만한 치료방법임을 보고하는 바이다.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.569-573
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• 1985

A 18-year-old female underwent surgical correction of tricuspid atresia and complete transposition of great arteries combined with atrial 8 ventricular septal defect and pulmonary stenosis. After the transection of main pulmonary artery just above the pulmonic valve, proximal portion of main pulmonary artery was closed with running suture and distal portion of main pulmonary artery anastomosed with right atrial appendage without valve insertion. Atrial septal defect was closed with running suture. Postoperative course was uneventful and she discharged on 18th postoperative day. Her condition is in very good until present. Modified Fontan`s operation without valve placement [in the condition of low pulmonary vascular resistance and good left ventricular function] may has a good result.

• Journal of Chest Surgery
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• v.51 no.1
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• pp.53-56
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• 2018

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adj acent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.618-626
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• 1986

Univentricular heart is a rare congenital cardiac anomaly in which the atrial chambers are connected to only one ventricular chamber and it consists of a diverse group of cardiac malformation characterized by both AV valves or a common AV valve opening into the same ventricle, or the presence of only a solitary AV valve. In spite of recent development in cardiac surgery, corrective operations for univentricular heart still have high mortality and complication rate. Twenty eight patients underwent corrective operation for univentricular heart at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital from February 1979 to July 1986. Of the 28 patients, 7 patients were operated on by ventricular septation and 21 patients by modified Fontan operation. Of the 28 patients, 19 patients were male and 9 patients female and ages ranged from 5 months to 18 years old with the average age of 7.3 years. There were 2 mortalities in 7 patients operated on by septation with the mortality rate of 28.6% and 5 complications, 3 complete AV block, 1 low cardiac output and 1 arrhythmia. All survived patients are being followed up without specific problem till now. There were 10 mortalities in 21 patients operated on by modified Fontan operation with the mortality rate of 47.6% and 10 complications, 2 low cardiac output, 2 respiratory failure necessitating tracheostomy, 2 persistent cyanosis, 2 arrhythmia, 1 missing of left AV valve in situs inversus patient due to misdiagnosis and one rupture of closed right AV valve. Incremental risk factors for operative mortality are young age less than 5 years old, anomalous pulmonary and systemic venous drainage and atrial septation procedure. In 11 survived patients, 9 patients show good follow-up results but one patient complains of persistent cyanosis and another one patient is suffered from CHF. In our series, results of corrective operation for univentricular heart shows continuing improvement but still high mortality and complication rate. So there must be continuing improvement in surgical result by selection of patient, by adequate decision making for timing and method of operation and by improving operative methods.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.48-51
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• 2002

The conversion of failing Fontan circuit to total cavopulmonary connection(TCPC) is recommended as a therapeutic option in patients with late Fontan complications such as atrial arrhythmia, atrial enlargement, pulmonary venous obstruction, and ventricular dysfunction. Combined TCPC with extracardiac conduit and cryoablation of arrhythmia circuit is preferred for treatment of failing Fontan coulection with atrial lachyarrhythrnia. We report a case of conversion of atriopulmonary connection to extracardiac conduit Fontan and cryoablation of atrial arrhythmia circuit in a patient with tricuspid atresia, who also had ectopic atrial tachycardia, right atrial thrombi, pulmonary venous obstruction, and ventricular dysfunction. This patient and the parents were Jehovah's Witnesses; therefore, the patient underwent the procedure without blood transfusion.