• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.308-311
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• 2005

The status of pulmonary circulation is regarded with utmost importance for the successful Fontan operation. The absence of unilateral pulmonary artery leads to decreased pulmonary vascular bed and elevated pulmonary vascular resistance which are the risk factors for Fontan operation. A 9-year-8-month-old female patient diagnosed as tricuspid atresia, pulmonary atresia with absent left pulmonary artery flow, received extracardiac conduit Fontan operation using 18 mm Gore-Tex graft. She was discharged on twenty sixth postoperative day with arterial $O_2$ saturation of $70\%$ on room air. On last follow up at 14 months after the operation, she was clinically well with $O_2$ saturation of $91\%$.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.50-57
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• 1986

Sixty-nine patients with various types of cyanotic congenital heart disease underwent systemic-pulmonary artery shunts with a microporous polytetrafluoroethylene [PTFE] prosthesis between 1979 and 1985. Their ages ranged from 2 months to 39 years [mean$\pm$SD: 5.2$\pm$7.4, median: 3.3 years]. Diagnosis included the following: Tetralogy of Fallot, 45: Double outlet right or left ventricle, 11: Single ventricle, .5: Transposition of great vessels, 4: Tricuspid atresia, 3 and Pulmonary atresia with intact ventricular septum, 1. Forty-eight patients had subclavian-pulmonary artery anastomosis, 12 patients aorta-right pulmonary artery anastomosis, 6 patients aorta-main pulmonary artery anastomosis, and 3 patients descending aorta-pulmonary artery anastomosis. The PTEE graft of 3 mm in diameter was used in 1, 4 mm in 29, 5 mm in 35 and 6 mm in 4 patients. Ten patients were died within 30 days after operation [mortality rate: 14.5%]. Among them, 6 patients were operated in urgency due to cardiac arrest or severe anoxic spell after cardiac catheterization, and so surgical mortality of elective operation is 9.5%. The 59 survivors showed improvement of the arterial oxygen saturation [65.4% - 9.8%] and hemoglobin [18.8 gm/dl - 16.0 gm/dl] values [V<0.01]. The follow up period ranged from 1 month to 67 months, [752 patient-months] and during this periods there were 4 late shunt failures after 3 months postoperatively with 4 mm graft, and 2 with 5 mm graft. The over-all patency rate of 4 mm PTFE was 85.9$\pm$9.2% [SEM] in 12 months and 40.9$\pm$22.5% in 24 months. The over-all patency rate of 5 mm PTFE was 87.5$\pm$9.6% in 12 months and 58.3$\pm$24.6% in 36 months. The lowest systolic pressure in death group was 64.9$\pm$15.0 mmHg and in survival group, 86.4$\pm$12.1 mmHg [P<0.001]. We think that the PTFE graft is useful in palliative shunt operation, but the effectiveness of the 4 mm PTFE graft may be limited. The blood pressure also may play an important role in patency of Prosthesis.

• Journal of Chest Surgery
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• v.38 no.6 s.251
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• pp.403-409
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• 2005

Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum, Material and Method: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days $(2\~382\;days)$. The average Z-value of the tricuspid annulus diameter was -3.1$(-5.6\~0.8)$. Thirteen $(36\%)$ patients had right ventricle-to-coronary artery fistulas, and $4(11\%)$ patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months $(3\;months\~2.2\;years)$, Result: Twenty-four patients under-went initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arierial shunt only (Z-value -2.2 vs. -4.8, p=0.000). There were $5(13\%)$ early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in $12(32\%)$ patients, single ventricular repair in $8(21\%)$, and one and a half ventricular repair in $4(l1\%)$ patients. Nine $(24\%)$ patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was $83.2\%$, respectively. Conclusion: Most of the deaths occurred after the initial palliation. Overall long-term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.

• Clinical and Experimental Pediatrics
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• v.61 no.7
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• pp.210-216
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• 2018

Purpose: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. Methods: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. Results: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1-98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2-31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. Conclusion: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.754-758
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• 1995

Between February 1987 and April 1994,30 modified Blalock- Taussing shunts[MBTS were carried out at the Department of Thoracic and Cardiovascular Surgery of the Keimyung University Dongsan Medical Center.The operation consists of interposing between the subclavian artery and the pulmonary artery a polytetrafluoroethylene graft.There were 19 boys and 11 girls.The average age at the time of shunt construction was 14 months [range 4 days to 5 years .Seventy-six percent [23/30 were less than 1 year of age.Cardiac defects treated with MBTS included tetralogy of Fallot[10 , pulmonary atresia with ventricular septal defect[8 , pulmonary atresia with intact ventricular septum[4 , uni-ventricular heart[3 , and other complex cardiac anomalies[5 .Prosthesis of 4mm were used in 13 cases, and 5mm in 17.Of the 30 operations, 21 were performed on the right side and 9 on the left side.The hemoglobin level decreased from 21.1 gm/dl preoperatively to 16.3 gm/dl postoperatively and systemic oxygen saturation level increased from 60.5 % preoperatively to 85.4 % postoperatively.In the 30 patients who recieved MBTS, there were one early [3% and three late deaths [10% .Seven patients have had an corrective operation and two patient required second palliative procedure.The remaining patients are awaiting further operation with ingestion of aspirin [5 mg/kg/day as an antiplatelet agent.These results indicate that the MBTS provide excellent palliation at a low operative mortality for most patients.

• 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.

• Clinical and Experimental Pediatrics
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• v.57 no.10
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• pp.461-463
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• 2014

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

• Journal of Chest Surgery
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• v.23 no.6
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• pp.1263-1269
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• 1990

Two patients with uncorrectable cyanotic cardiac anomalies underwent total cavopulmonary shunt[modified Fontan operation]. Case I was a 14 years old male with dyspnea and cyanosis after birth. Aortogram showed TGA combined with overriding of aorta, pulmonary stenosis, complete atrioventricular septal defect, interruption of inferior vena cava, and situs inversus totalis. We had performed total cavopulmonary shunt using with 16 mm Gortex Graft in single atrium to bypass the hepatic vein to pulmonary artery. Postoperatively, patient sustained low PaCO2 and low cardiac output and then expired at 19th postoperative day. The cause of death of the patient would be low cardiac output. Case II was a 6 years old female with dyspnea and cyanosis after birth. Aortogram showed tricuspid atresia[Type IIb], transposition of great arteries, atrial septal defect, ventricular septal defect and pulmonary stenosis, We had performed total cavo-pulmonary shunt using intraatrial baffle[tunnel] with Goretex patch. The postoperative course of this patient was good without event.

• Journal of Chest Surgery
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• v.33 no.12
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• pp.959-962
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• 2000

A 4-year and 11-month old child was diagnosed as having dextrocardia, pulmonary atresia, atrioventricular discordance, aorta from right ventricle, PDA, the interruption of right pulmonary artery and postoperative state of Blalock-Taussing shunt on right pulmonary artery. Anatomical repair so called \"double switch operation\" was performed; the Ratelli procedure on ventricular level and the Mustard procedure on atrial level. We report the successful anatomical repair of congenitally Physiologically Corrected Transposition even with Dextrocardia, Situs Inversus and the interruption of Right Pulmonary Artery.ry Artery.

• Journal of Chest Surgery
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• v.33 no.6
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• pp.476-486
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• 2000

Background: Pulmonary atresia with intact ventricular septum(PA/IVS) is an anatomically heterogenous anomaly with a variety of surgical strategies possible. The purpose of the study is to evaluate the influence of right ventricular size on the early and midterm results of surgical repair of PA/IVS. Material and method: Medical records of 20 consecutive patients with PA/IVS operated on between January 1993 and August 1999 were retrospectively reviewed. There were 12 boys and 8 girls whose ages ranged from 2 days to 14.5 months (median 6 days). Their body weight ranged from 2.52kg to 9.35 kg(median 3.18kg). The preoperative Z-value of the diameter of the tricuspid valve(T-valve) was less than or -4 in 5 patients, between -4 and -2 in 1, between -2 and 0 in 7, between 0 and 2 in 6, and greater than or 2 in 1. All patients who had z-value of tricuspid valve greater than -2.05 were attempted biventricular repair(n=15) and all patients who had it smaller than -4.4 underwent systemic-pulmonary shunt operation only(n=3) or bidirectinal cavopulmonary shunt with right ventricular reconstruction(n=2). Result: Two early deaths(2/20, 10%) occurred. Both were infants who underwent transannular patch with shunt. One of these two had huge right ventricle(Z-value of tricuspid valve = 5). There were 2 late non-cardiac deaths 3 and 7 months after operations respectively. Follow-up was completed in all children at a mean of 35.3 months(range, 5 to 54 months). 10 of 11 survivors who underwent transannular patch or valvotomy with or without shunt procedure were in NYHA functional class I even though some of them had small interatrial communication or patent shunt. All three patients who had shunt procedure only at initial palliation completed Fontan procedures with no death. Two patients who underwent right ventricular outflow reconstruction with bidirectional cavopulmonary shunt were also in good condition. Conclusion: The transanular RVOT patch or valvotomy with or without systemic-pumonary shunt as an initial palliative procedure to achieve biventricular repair for the patients who had neither too small nor too large right ventricle(-2.05$\leq$Z-value of T-value of T-valve$\leq$2) could be performed at low operative risk(1/14 7.1%). Systemic-pulmonary shunt procedure and bidirectional cavopulmonary shunt procedure for the patients who had small right ventricle(Z-value of T-valve$\leq$4.4) could be also performed with low risk. But a patient with huge right atrium and ventricle(Z-value of t-valve=5) had poor operative result.