• 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.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.

• 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.26 no.5
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• pp.422-426
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• 1993

We report three cases of children who underwent definitive conversion to the Fontan circulation using a new surgical technique, Extracardiac Epicardial Lateral Tunnel. This new procedure allows the operation to be performed as a totally extracardiac operation [especially in ventricular dysfunction] and allows it to be performed in a very small atrium and in cases with unsuitable pulmonary venous drainage.Our data suggest that this procedure may achieve satisfactory hemodynamics of the total cavopulmonary connection.

• 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.

• 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.50 no.3
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• pp.207-210
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• 2017

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

• 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.18 no.4
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• pp.549-556
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• 1985

Since the first report on a successful method of correcting tricuspid atresia by Fontan and Baudet in 1971, several alternative surgical techniques have been described. Bjrk`s modification was performed three patients, 3, 8, and 9 years of age, in our Hanyang University Hospital. The diagnoses were tricuspid atresia type lb, tricuspid atresia type lib and d-TGA with huge VSD and valvular PS. None had previous palliative procedures. The patients were operated on with good results. The CVP after operation was 10-14 mmHg and early extubation was done. Postoperatively, none had peripheral edema or ascites. Postoperatively, all three were acyanotic and had improved physical capacity compared with their preoperative status.

• 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.