• 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.40 no.12
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• pp.805-810
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• 2007

Background: This paper reviews our experience retrospectively to examine the clinical results and effectiveness of lateral tunnel (LT) and extracardiac conduit (ECC) Fontan procedures at a single institution. Material and Method: One hundred and sixty five Fontan procedures were performed (67 LT and 98 ECC) between January 1996 and December 2006. Preoperative and postoperative hemodynamic values, arrhythmia, hospital and intensive care unit stay, chest tube drain, morbidity and mortality were reviewed. Result: The overall operative mortality in the LT and ECC groups was 4.5% (3) and 2.0% (2), respectively. There was a significant difference in the immediate postoperative transpulmonary gradient (LT $8.5{\pm}2.5$ vs ECC $6.6{\pm}2.4$, p-value<0.001) and central venous pressure (LT $18.3{\pm}3.8$ vs ECC $15.6{\pm}2.4$, p-value=0.001) between the two groups. The mean follow-up in the LT and ECC groups was $74.1{\pm}31.5$ and $38.1{\pm}29.1$ months, respectively. There was one late death. The actuarial survival at 10 years in the LT and ECC groups was 92% and 89%, respectively. In arrhythmia, the ECC patients showed a slightly low incidence but the difference was not statistically significant. Conclusion: Both the LT and ECC Fontan procedures showed comparable early and mid-term outcomes in terms of the surgical morbidity and mortality, postoperative hemodynamics, and mid-term survival. The ECC Fontan procedure reduces the risk of arrhythmia in the follow up period.

• Journal of Chest Surgery
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• v.34 no.10
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• pp.781-783
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• 2001

In a patient with single ventricle associated with complex systemic and/or pulmonary venous drainage, intraatrial Fontan procedure is sometimes technically difficult due to the complex spatial relationship between their orifices in the atrium. We report a case of the modified extracardiac conduit Fontan procedure in a patient with a single ventricle in which the inferior vena cava and the hepatic vein drained separately into the atrium and the intraatrial orifice of the hepatic vein was abut to the orifice of the left lower pulmonary vein.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.457-462
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• 2003

Background: Surgical results of the Fontan procedures in patients with a single ventricle have improved. As the perioperative mortality continues to decline and late outcome is forthcoming, attention is now being directed toward late complications of the Fontan procedures. We retrospectively analyzed our experience with reoperations after Fontan procedures. Material and Method: Between January 1988 and December 2002, 24 patients underwent reoperations after Fontan procedures. The median age at Fontan procedures and reoperation was 3.3 years and 9.2 years, respectively. Types of initial Fontan procedures were atriopulmonary connection (n＝11), lateral tunnel Fontan (n＝11), and extracardiac conduit Fontan (n＝2). Indications for reoperation included atrioventricular valve regurgitation (n＝7), atrial arrhythmia (n＝8), Fontan pathway stenosis (n＝7), residual right-to-left shunt (n＝5), etc. Result: Procedures performed at reoperation included atrioventricular valve replacement (n＝6), conversion to lateral tunnel Fontan (n＝5), conversion to extracardiac conduit Fontan (n＝3), cryoablation of arrhythmia circuit (n＝7), etc. There was no operative mortality. There were 2 late deaths. Mean follow-up duration was 2.7$\pm$2.1 years. All patients except two were in NYHA class I at the latest follow-up. Among 8 patients with preoperative atrial arrhythmia, postoperative conversion to normal sinus rhythm was achieved in 7 patients. Conclusion: Reoperations after Fontan procedures could be achieved with low mortality and morbidity. Reoperation may lead to clinical improvement in patients with specific target conditions such as atrioventricular valve regurgitation, refractory atrial arrhythmia, or Fontan pathway stenosis, especially in patients with previous atriopulmonary connection.

• Journal of Chest Surgery
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• v.37 no.8
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• pp.644-651
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• 2004

Background: Refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with arrhythmia surgery in patients who had Fontan operation. Material and Method: Between July 1986 and December 2003, 275 early survivors after Fontan operation were reviewed. Fourteen patients underwent. arrhythmia surgery at reoperation after Fontan operation, and mean age at reoperation was 16.8$\pm$7.1 (range: 4.5 ∼ 30.6) years. Mechanisms of arrhythmia included atrial flutter in 8 patients, and atrial fibrillation in 2. Arrhythmia surgery has evolved from isthmus cryoablation in 12 patients to right-sided maze in 2 patients. Thirty-two patients. underwent prophylactic isthmus cryoablation concomitantly at initial Fontan operation. Result: Postoperative arrhythmias occurred in 68 patients (24.7%) among 275. There was no early and late mortality after the arrhythmia surgery. After redo Fontan operation, all patients maintained normal sinus rhythm. Atrial flutter recurred in 3 patients who had sinus conversion with medication and 7 required permanent pacemakers with a mean follow-up of 26.5$\pm$29.1 (range: 2 ∼ 73) months. All patients have improved to NYHA class I or II. After prophylactic cryoablation at initial Fontan operation, 29 patients (90.6%) had sinus rhythm, 1 patient had junctional tachycardia, 1 patient had sinus nodal dysfunction, and 1 patient had AV block with a mean follow-up of 51.3$\pm$19.8 (range: 4∼80) months. Conclusion: Redo Fontan operation, and concomitant arrhythmia surgery reduced atrial arrhythmias and improved NYHA functional classification.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.559-565
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• 2003

By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and method: From October1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0$\pm$7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was 111 or IV in six patients. Paroxysmal atrial fibrillation (n:f), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional lachyarrhythrnia (n=1). Postoperative central venous Pressure was lower than the preoperative value (17.9$\pm$3.5 vs. 14.9$\pm$1.0, p=0.049). Follow-up was complete in all patients and extended to 50,1 months (mean, 30.3$\pm$ 12.8 months). There was no late death. All patients were in NYHA class 1 or 11. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure, PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

• Journal of Chest Surgery
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• v.35 no.2
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• pp.137-140
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• 2002

Aortic regurgitation in the pediatrics who had previous cardiac surgery is increased with their improved longterm survival rate and their complexity of heart disease. So the need of aortic valve surgery in pediatrics is also on the increase. A 10-year old boy was admitted for progressive cyanosis and dyspnea on exertion(DOE). The patient had been underwent lateral tunnel Fontan operation before. Echocardiography and cardiac catheterization study revealed hepatic vein drained to pulmonary atrium via intrahepatic collaterals, moderate atrioventricular regurgitation, and severe aortic regurgitation due to aortic root dilation. We report a case who had aortic root reconstruction, valvuloplasty of the atrioventricular valve, and hepatic vein ligation successfully Cyanosis and DOE was dramatically improved after the operation

• Journal of Chest Surgery
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• v.41 no.3
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• pp.305-312
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• 2008

Background: Anatomic and functional abnormalities of the systemic atrioventricular (AV) valve are common in single ventricle. pathologies and continue to be associated with poor early and late outcomes in surgically palliated single. ventricle patients. We aggressively performed valvuloplasty for atrioventricular valve regurgitation (AVVR) during the course toward a Fontan operation. Material and Method: Between January 1995 and December 2004, 209 patients underwent a Fontan operation in our institution. We retrospectively evaluated the prevalence of AVVR and the influence of AV valve repair on outcome, and we analyzed the progression of AVVR after the Fontan operation for 168 patients where echocardiographic follow up results for more than 6 months after the Fontan operation were available. During the course toward a Fontan operation, 25 patients underwent 30 procedures for AVVR. These procedures. were. carried out during placement of a bidirectional cavopulmonary shunt (BCPS) for nine patients, between the time of placement of a BCPS and the Fontan operation for four patients, and during the Fontan operation for 17 patients. Five patients underwent procedures for AVVR twice. Result: The late mortality rate after the Fontan operation was 4.2% (n=7), with a median follow-up duration of 52 months (range, $6{\sim}123$ months). Seven patients (4%) had unfavorable outcomes such as significant (moderate or severe) AVVR in six patients, and significant AV valve stenosis in one patient was determined at the last follow up after the Fontan operation. Among the seven patients, four patients underwent AV valve repair after the Fontan operation, and one patient underwent subsequent AV valve replacement. Progression to AVVR of equal to or greater than grade 2 was noted in 30 patients (18%) at the last follow up after the Fontan operation, including 12 patients that underwent previous AV valve procedures. Initial grading of AVVR, a previous AV valve operation, and specific AV valve morphology such as a common AV valve or mitral atresia were significant risk factors for the progression of AVVR after the Fontan operation. Conclusion: In our surgical series, a small percentage of patients showed unfavorable outcomes. related to AVVR during the course toward a Fontan operation. However, a closer follow-up is required to evaluate the progression of the AVVR after a Fontan operation, especially for patients showing poor AV valve function at the first presentation and specific AV valve morphology.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.439-448
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• 2002

Pulmonary artery banding (PAB) in the functional univentricular heart (UVH) is a palliative procedure for staging toward the Fontan procedure; however, it is known to be a risk factor. Material and method： The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively. We investigated the aortic arch obstruction, the development and progression of subaortic stenosis after PAB, and risk factor of mortality according to surgical method. Result： In 37 neonates and infants with single ventricular physiology, aortic arch obstruction was combined in 7. There were 6 early deaths (16.2%) after PAB and 3 late deaths (8.1%) after Fontan operation. The actuarial overall survival including early mortality at 3 and 5 years were 80.7$\pm$6.6%, 72.2$\pm$8.2% respectively. Among 31 patients who survived PAB, 27 patients (87.1 %) could become candidates for Fontan operation； 22 patients (71.0%) completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS) or Fontan operation (follow-up mean 4.5 year, minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29, 27.6%)； 3 cases in the patients without arch anomaly (3/22, 13.6%) and 5 in those with arch anomaly (5/7, 71.4%). The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS) in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p＜0.001). The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). Conclusion： PAB is effective as an initial palliative step in functional UVH. And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step, with subsequent DKS for subaortic stenosis. This strategy, initial PAB and careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.40-46
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• 2007

Purpose : This study assessed the long term survival rate and long term complications of patients who had a modified Fontan operation for functionally univentricular cardiac anomaly. Methods : Between June 1986 and December 2000, 302 patients with a functional single ventricle underwent surgical interventions and were followed up until February 2006. The mean follow-up period was $8.3{\pm}5.3years$ (range 3.5-18 years). Their median age was 2.4 years at the Fontan operation. The survival rate, the incidence and the risk factor of late complications were evaluated retrospectively. Results : The verall survival rate was 91 percent at 5 years and 87 percent at 10 years. In multivariate analysis, early calendar year of operation and significant regurgitation were risk factors of death. The surviving patients showed NYHA functional class I in 82 percent, class II in 15 percent, and class III in 3 percent. Redo Fontan operations were necessary in 8.8 percent of patients at average $12.8{\pm}3.6years$ after initial Fontan operation. The most common cause of Fontan conversion was atrial arrhythmia. The incidence of thromboembolic events was 9.3% and these complications were associated with the occurrence of atrial tachyarrhythmia. Supraventricular tachycardia including atrial flutter or fibrillation were reported on the follow-up examination by 11.2 percent of survivors after $8.4{\pm}5.6years$. Atriopulmonary connection showed higher rates of late tachycardia than lateral tunnel operation. Conclusions : This study revealed that the recent survival rate of Fontan type operation was satisfactory, but the occurrence of late complications after a Fontan type operation increased with the longer survival. There is a need for strict follow up and early treatment of late complications in patients who had a Fontan operation.