• Journal of Chest Surgery
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• v.37 no.1
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• pp.72-75
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• 2004

The Fontan operation is commonly practiced for the physiologic correction of univentricular heart diseases. However, for the patients who have risk factors against this operation, it is recommended to take the initial palliative operation rather than going to the Fontan operation at once. The proper timing to the Fontan operation after palliation is decided by assessing several factors such as patient's age and other risks of maintaining palliative state, etc. Usually, the Fontan operation is done relatively early after palliation stage. Here, we report a 36 years old-adult-female with univentricular heart disease who underwent the successful Fontan operation at 17 years after unidirectional Glenn procedure.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.539-542
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• 2004

Not only morphology of pulmonary artery or pulmonary artery resistance, but major aortopulmonary collateral arteries are risk factors of Fontan procedure. We report a successful Fontan procedure after rehabilitation of pulmonary arteries by unifocalization and systemic to pulmonary shunt in a high risk Fontan candidate with functional single ventricle combined with hypoplastic pulmonary arteries and major aortopulmonary collateral arteries supplying most of the bilateral lung field.

• Journal of Chest Surgery
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• v.36 no.2
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• pp.73-78
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• 2003

The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. Material and Method: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8 :-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1 Result: There were 3 early and late deaths respectively Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1) In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. Conclusion: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However, more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class, Although the results were not satisfactory enough in all patients.

• 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.42 no.1
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• pp.9-13
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• 2009

Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1062-1068
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• 1997

From August 1989 to January 1996, a total of 105 cases of bidirectional Glean operations have been done as the interim stage for the patien s with some risk of univentricular correction at Sejong General Hospital. From December 1992, we started the conversion to Fontal operations for them, and 42 cases underwent Fontal-stage operation till February 1996. Their diagnoses were univentricular heart in 19(right ventricular type : 14), tricuspid atresia 11, double outlet of right ventricle 9, and others in 3 cases. The median age of bidirectional Glerln-stage operation was 12.5 months(range 2 months to 8 years) and Fontan-stage operation was at 59.6 months of median age(range 1 year 5 months to ,9 year 7 months). The mean waiting interval between the two operations was 33.88 $\pm$ 17.85 months with a range of 10 months to 6 years 3 months. During the waiting periods, 18 patients developed significant systemic-pulmonary collaterals andfor systemic verso-veno collateral channels. There were 5 hospital deaths after operations due to low cardiac output in 4 and sepsis in one. Most of the Fontal-stage operations were done by the late al tunneling with Core-Tex tube graft patch and fenestrated with the size of 2.5 ~6 mm. All the patients were followed-up(7 months to 4 years 2 months, mean 21.97$\pm$10.82 months) and there were 5 late deaths(postoperatively 6 months to 2 years) due to thromboembolism in 1, after heart transplantation 1, plastic bronchitis 1, protein loosing enteropathy 1, and pneumonia in 1. Dividing the patients by the waiting interval of 2 years, the early correction to Fontal group (N=16) showed the better results(hospital mortality 1116, late mortality 1116, significant collateral development 2/16) compared to the other group(N=26) (4/26, 4/26, 16/26). In conclusion, after the bidirectional Glean-stage operation successfully got rid of the previous risk factors, we recommand to do the Fontan-stage operation no later than 2 years of interval.

• 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.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.23 no.5
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• pp.936-943
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• 1990

The Fontan operation for tricuspid atresia was first performed in 1968[Fontan and Baudet 1971] and several technical modifications of procedure were developed. We have experienced a case of modified Fontan operation for tricuspid atresia type Ic who was 5 years old female. She had previous palliative procedure \ulcornerpulmonary artery banding due to excessively increased pulmonary blood flow. The modified Fontan operation was right atrium to right ventricle connection with valve-bearing conduit. The result was good and the patient was discharged 20 days after operation.

• 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.