• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.529-537
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• 2005

Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.177-181
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• 2002

Background: Follow-up studies have shown that although outcomes have improved substantially over time, results of the Fontan operation and its modifications remain suboptimal. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus early and midterm change of internal diameter of PTFE conduit. Material and Method: Between April 1997 and July 2000 were reviewed. Twelve patients (M:6, F:6, mean age 42.04 $\pm$ 12.43months, mean body weight 13.80$\pm$ 1.94kg) underwent extracardiac conduit Fontan operation with expanded PTFE graft. Mean cardiopulmonary bypass time was 109.7$\pm$26.99minute and mean operation time was 455$\pm$89.51minute. Intraoperative fenestration was performed in 10 patients. The aortic cross clamping was not performed in all patients. Result: There was no early deaths and no postoperative dysrhythmia. Postoperative protein losing enteropathy and prolonged pleural effusion occurred in 1(8.3%) and 4 patients(33.3%). Conduit patency was evaluated by magnetic resonance imaging studies. A 9.84$\pm$3.84% mean reduction in conduit internal diameter and there was no statistical correlation between the change of internal diameter of conduit and the postoperative duration after partial correlation analysis(r=0.019, p=0.955). Conclusion: These results demonstrate that the extracardiac conduit Fontan operation provies good early and midterm results and may reduce the prevalence of late arrhythmia. And there is no correlation between the change of internal diameter of conduit and the postoperative duration after extracardiac conduit Fontan operation with the expanded PTFE graft conduit.

• Proceedings of the KTCVS Conference
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• 2004.06a
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• pp.153-154
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• 2004

• Proceedings of the KTCVS Conference
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• 1997.04a
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• pp.74-79
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• 1997

• Proceedings of the KTCVS Conference
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• 1997.04a
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• pp.92-102
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• 1997

• Proceedings of the KTCVS Conference
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• 1997.04a
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• pp.80-91
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• 1997

• Proceedings of the KTCVS Conference
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• 1998.10a
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• pp.137-137
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• 1998

• Proceedings of the KTCVS Conference
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• 1993.06a
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• pp.43-43
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• 1993

• 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.643-648
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• 1985

Tricuspid atresia is the third most common cause of cyanotic heart disease, following T.O.F. and T.G.A. It is seen in about 30% of patients with congenital heart disease on postmortem examination. Recently, we experienced a case of tricuspid atresia, who had received Blalock-Taussig Shunt operation 10 years ago. The Patient was 15 year-old girl with the complaints of persistent cyanosis and exertional dyspnea. Cineangiography revealed Keith type lib tricuspid atresia, so, RA appendage was anastomosed to the right pulmonary artery and ASD was also closed using patch. Postoperative course was very difficult because of persistent right heart failure and weak respiratory power, but from postoperative 15th day, all cardiac and respiratory problems were resolved. We followed up this patient for about 1 year, and her condition is excellent up to now.