• Journal of Chest Surgery
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• v.51 no.1
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• pp.53-56
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• 2018

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adj acent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.472-480
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• 1998

We reviewed the surgical results of intracardiac lateral tunnel Fontan procedure for the repair of functional single ventricles. Between 1990 and 1996, 104 patients underwent total cavopulmonary anastomosis. Patients' age and body weight averaged 35.9(range 10 to 173) months and 12.8(range 6.5 to 37.8) kg. Preoperative diagnoses included 18 tricuspid atresias and 53 double inlet ventricles with univentricular atrioventricular connection and 33 other complex lesions. Previous palliative operations were performed in 50 of these patients, including 37 systemic to pulmonary artery shunts, 13 pulmonary artery bandings, 15 surgical atrial septectomies, 2 arterial switch procedures, 2 resections of subaortic conus, 2 repairs of total anomalous pulmonary venous connection and 1 Damus-Stansel-Kaye procedure. In 19 patients bidirectional cavopulmonary shunt operation was performed before the Fontan procedure and in 1 patient a Kawashima procedure was required. Preoperative hemodynamics revealed a mean pulmonary artery pressure of 14.6(range 5 to 28) mmHg, a mean pulmonary vascular resistance of 2.2(range 0.4 to 6.9) wood-unit, a mean pulmonary to systemic flow ratio of 0.9(range 0.3 to 3.0), a mean ventricular end-diastolic pressure of 9.0 (range 3.0 to 21.0) mmHg, and a mean arterial oxygen saturation of 76.0(range 45.6 to 88.0)%. The operative procedure consisted of a longitudinal right atriotomy 2cm lateral to the terminal crest up to the right atrial auricle, followed by the creation of a lateral tunnel connecting the orifices of either the superior caval vein or the right atrial auricle to the inferior caval vein, using a Gore-Tex vascular graft with or without a fenestration. Concomitant procedures at the time of Fontan procedure included 22 pulmonary artery angioplasties, 21 atrial septectomies, 4 atrioventricular valve replacements or repairs, 4 corrections of anomalous pulmonary venous connection, and 3 permanent pacemaker implantations. In 31, a fenestration was created, and in 1 an adjustable communication was made in the lateral tunnel pathway. One lateral tunnel conversion was performed in a patient with recurrent intractable tachyarrhythmia 4 years after the initial atriopulmonary connection. Post-extubation hemodynamic data revealed a mean pulmonary artery pressure of 12.7(range 8 to 21) mmHg, a mean ventricular end-diastolic pressure of 7.6(range 4 to 12) mmHg, and a mean room-air arterial oxygen saturation of 89.9(range 68 to 100) %. The follow-up duration was, on average, 27(range 1 to 85) months. Post-Fontan complications included 11 prolonged pleural effusions, 8 arrhythmias, 9 chylothoraces, 5 of damage to the central nervous system, 5 infectious complications, and 4 of acute renal failure. Seven early(6.7%) and 5 late(4.8%) deaths occured. These results proved that the lateral tunnel Fontan procedure provided excellent hemodynamic improvements with acceptable mortality and morbidity for hearts with various types of functional single ventricle.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

• 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.20 no.1
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• pp.22-29
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• 1987

From Sep. 1978 to Aug. 1986, 44 cases of Fontan operation were performed at Seoul National University Hospital. 1] The diagnoses were TA in 13 [38.6%], UVH in 21 [47.79`], DORV in 3 [6.8%], TGA in 2 [4.5%] and C-ECD with DORY in 1[2.3%]. 2] There were 20 operative deaths [44.5%]. 3] The operative risk factors were early date of operation between 1978 and 1983, young age below 3 years old, direct atriopulmonary anastomosis without roofing, and postoperative high CVP above 25cmH,O. But the relation between operative mortality and various cardiac diseases was absent. 4] survived patients were followed from 1 to 54 months except 3 patients who were lost to follow up. 16 patients were in functional class I and 1 in class II, 2 of the above 17 patients were reoperated due to residual right to left shunt. In remained 4 patients, 3 patients persisted cyanosis after operation and 1 patients died 1 month postoperatively due to pulmonary embolism. 5] As 4 result, the Fontan procedure can be done with a good result for tricuspid atresia and other complex lesions. The operative mortality can be reduced further with a correct anatomical diagnosis preoperatively, rigid operative criteria to pulmonary vascular resistance, direct atriopulmonary anastomosis with roofing, and use of `Venous Assist Device` postoperatively in low cardiac output patients.

• 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.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.51 no.3
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• pp.167-171
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• 2018

Background: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half ($1\text\tiny{1/2}$) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. Methods: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or $1\text\tiny{1/2}$ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3-24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. Results: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54-68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62-128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. Conclusion: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or $1\text\tiny{1/2}$ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

• 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.17 no.4
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• pp.625-631
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• 1984

A modified Fontan procedure was performed on two patients with Univentricular heart. The first patient had UVH of right ventricular type with trabecular pouch and had various associated anomalies, such as common atrium, common atrioventricular valve and combined pulmonary stenosis. The second patient had UVH of left ventricular type with outlet chamber and the associated anomalies were atrial septal defect, tricuspid stenosis and combined pulmonary stenosis. Postoperative hemodynamic insufficiency, fluid retention and renal insufficiency were occurred in the first patient, but relieved with the aid of inotropics and vasodilators. We thought that the good postoperative course and surgical result were gained from the widely patent atriopulmonary anastomosis.