• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.281-288
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• 2006

Background: Extracardiac pericardial-flap lateral tunnel Fontan operation has theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. Material and Method: Clinical data was reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was $2.8{\pm}1.5$ years and the body weight was $12.3{\pm}3.2$ kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms, the diameter and the cross-sectional area of the lateral tunnel was compared to those of inferior vena cava. Result: There were four operative mortality cases (9.8%) and the causes of death were low cardiac output for all four cases. Postoperatively, five patients had prolonged pleural effusion longer than two weeks and one patient required a permanent pacemaker due to complete heart block. Follow-up was possible in 37 patients and the follow up duration was $3.8{\pm}2.2$ years. During that period, one patient died, of upper gastrointestional bleeding combined with heart failure and one patient died a sudden death of unknown cause. Two patients required reoperation due to subaortic stenosis and anastomosis site stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was anew but there was no thromboembolic complication. The lateral tunnel showed growth in proportion to the size of the inferior vena cava. Conclusion: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and safe. The mid-term result was favorable and the extracardiac tunnel showed potential for growth.

• 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.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.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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• 1993.06a
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• pp.43-43
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• 1993

• Journal of Chest Surgery
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• v.20 no.3
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• pp.536-543
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• 1987

The Fontan procedure was physiological correction which was initially applied to tricuspid atresia. We had used the modified Fontan operation in 8 cases at National Medical Center, Seoul, from Aug. 1984 to Oct. 1986. Age range was 20 months to 15 years [mean: 9 years] and male: female ratio was 5:3. 5 patients had tricuspid atresia [lb: 2 cases, Ic: 1 case, lib: 1 case, & llc: 1 case], 2 Patients had univentricular heart of left ventricular type, and one patient had transposition of great arteries with complete endocardial cushion defect. The operative principle was direct anastomosis between right atrium and pulmonary artery, whether main pulmonary artery or right pulmonary artery without any conduits. Postoperatively all patients needed high central venous pressure for adequate hemodynamic status in both survival [20-24 CmH2O] and mortality groups [20-24 CmH2O]. The fatal complications were as follows: empyema with bronchopleural fistula [1 case], bleeding tendency & brain damage [1 case], low cardiac output syndrome & acute renal failure [2 cases], and right to left shunt of unknown origin [1 case]. There were 5 hospital deaths; 3 of 5 tricuspid atresia patients, 1 of 2 univentricular heart patients, and 1 of 1 transposition of great arteries with complete endocar4ial cushion defect patient. The overall mortality was 62.5%. 3 survivors were nearly compatible with Choussat & Fontan criteria. Thus appropriate patient selection and experienced surgical technique were required for good results.

• Journal of Chest Surgery
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• v.22 no.1
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• pp.25-31
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• 1989

Forty-five patients, aged 16 months to 15.5 years mean 69 months, with a wide variety of cardiac malformations underwent the modified Fontan operation between Sep. 1986 to Aug. 1988. Nineteen patients had previously undergone palliative operations mainly modified B-T shunt. Twenty patients had a mean pulmonary artery pressure greater than 15 mmHg, with nine operative deaths. Thirteen patients had anomalies of systemic venous connection and seven patients had anomalies of pulmonary venous connection. There were eighteen patients under the age 4 years and fifteen of them survived [83.3%]. Eighteen patients had a pulmonary vascular resistance [PVR] more than 2.5U/m, and nine died [50%] whereas two of twenty-three with a PVR less than 2.5U/m died[8.6%]. PVR and anomalies of pulmonary venous connection had a significant influence on survival, but age and anomalies of systemic venous connection did not. Amount of pleural effusion drained postoperatively and PVR had positive linear correlation. Pulmonary artery pressure was not an independent predictor of outcome and pulmonary artery pressure alone should not contraindicate a Fontan procedure if PVR is low. In general, the Fontan operation should be done at a younger age less than 4 year to avoid ventricular dysfunction due to long-standing exposure to hypoxia.

• 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.

• 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.21 no.5
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• pp.948-953
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• 1988

A technique applicated for physiologic correction of complex congenital cardiac disease suitable for Fontan procedure in which drainage of left superior vena cava and hepatocardiac vein to left atrium combined is described. We made one systemic venous baffle from left hepatocardiac vein to left superior vena cava and another systemic venous baffle from right inferior vena cava to the right superior vena cava with rigid prosthetic material[0.5mm thickness PTFE patch]. And then we anastomosed directly between the right sided atrial appendage and right pulmonary artery, and left-sided atrial wall beneath the appendage and left pulmonary artery. We believe that this procedure is superior to the method using intraatrial tube graft to divert the left hepatocardiac venous blood to right atrium, and applicable for physiologic correction of any complex congenital cardiac disease suitable for Fontan-type procedure in which anomalies of systemic venous drainage combined.