• Journal of Chest Surgery
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• 제25권4호
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• pp.406-411
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• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• Journal of Chest Surgery
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• 제35권2호
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• pp.137-140
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• 2002

선천성 심기형 환아들의 장기생존이 향상되면서 소아연령에서의 대동맥판막의 문제는 드물지 않게 경험하게 되며 또한 점차 증가하고 있다. 따라서 소아연령에서도 대동맥판막수술의 필요성이 점차 증가하고 있다. 과거 심장외측통로폰탄술식을 받은 10세 환아가 점차 심해지는 청색증과 운동시 호흡곤란으로 내원하였다. 술전 시행한 심초음파 및 심도자검사에서 간내 측부순환에 의해 간정맥에서 폐 심방으로 체정맥혈류가 유입되고 있었으며 방실판막은 중등도의, 상행대동맥의 확장 및 대동맥륜의 확장으로 인한 대동맥판막의 심한 폐쇄부전을 동반하고 있었다. 수술은 먼저 폐 심방으로 연결되는 간정맥을 결찰하고 체외순환을 가동하고 심정지를 시킨 후 상행대동맥의 축소성형 및 대동맥등 축소화를 포함한 대동맥근 재건술및 방실판막에 대한 성형술을 시행하였다. 술후 환아의 호흡곤란 및 청색증은 상당히 개선되었다.

• Journal of Chest Surgery
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• 제36권4호
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• pp.280-284
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• 2003

양방향 상행대정맥-폐동맥 단락술 후 폰탄 수술의 위험이 큰 2명의 환자에서 청색증의 증가와 운동 능력의 감소를 보여 액와동정맥루 형성술을 시행하였다. 수술 후 두 환자 모두 수술 후 1일째 일반 병실로 전원되었으며, 수술 후 각각 4일, 3일에 퇴원하였다. 두 증례 모두 수술 후 volume loading의 소견은 없었고, 8개월째 추적 관찰 중이며 청색증의 감소와 운동 능력의 향상을 관찰할 수 있었다. 그러나 수술 후 6개월째 시행한 폐관류스캔이나 contrast심초음파 검사에서 폐동정맥루가 아직 충분히 경감되지 않아, 향후 이에 대한 면밀한 추적 관찰이 필요할 것으로 생각한다.

• Journal of Chest Surgery
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• 제30권3호
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• pp.263-269
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• 1997

1987년 4월부터 1996년 5월까지 복잡 선천성 심기형을 가진 13명의 환자에서 Norwood술식을적용 하였다. 진단별 분포로는 좌심형성부전증후군이 7례이었고, 상행대동맥과 대동맥궁의 형성부전이 있으 면서 심실중격결손증 및 대동맥하 협착증을 동반한 승모판 협착증, 심실중격결손증, 대동맥축착 및 대 동맥하 헙착증을 동반한 승모판 폐쇄증, 심실중격결손증 및 대동맥하 협착증을 동반한 대동맥궁 단절, 대혈관전위를 동반한 삼첨판 폐쇄증이 각각 1례씩 있었으며 이중입구부 좌심실이 2례 있었다 수술 당 시 나이는 3일에서 8.7개월 (평균 60.5 $\pm$ 71.6일, 중앙값 i9일)이었다. 수술 사망율( <30일)은 46% (6명) 이었으며 만기 사망율은 15% (2명)였다. 모든 수술사망은 술후 24시간 이내에 발생하였으며 체외순환으 로부터 이탈에 실패한 경우가 5례, 술후 갑작스런 혈역학적 불안정으로 사망한 경우가 1례 있었고, 만기 사망은모두 흡인성 폐렴에 의한 것이었다. 5명의 장기 생존자에 대한추후술식으로는2명의 환자에서 Norwood술식 후 각각 12, 17개월에 변형 Fontan수술을 하였으며 1명에서 4.5개월 후에 완전 대정맥폐동맥 단락술을 시행하였고 수술 사망은 없었다. 나머지 2명의 환자에서는 Fontan 술식전의 중간단계 로 양방향성 상대정맥-폐동맥 단락술을 각각 3, 5.5개휠 후에 시행하였으며 수술사망이 1례 있었다. 수 술 사망 및 만기 사망을 포함한 모든 환자에서 1년 생존율은 30.8%였다. 결론적으로 본원에서 경험한 Norwood 술식의 사망율이 다른 주요 심장기 형의 사망율과 비교하여 높지만 이에 대한 경험이 축적되면서 더 좋은 성적을 기대할 수 있을 것으로 생각된다.

• Journal of Chest Surgery
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• 제20권4호
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• pp.751-759
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• 1987

We have experienced 4 cases of modified Fontan operation for Tricuspid atresia type III from February 1983 to May 1987 at the department of Thoracic 8 Cardiovascular Surgery, Seoul National University Hospital. There were 3 cases with left juxtaposition of right atrial auricle. The operatic mortality rate was 25% [one case death].

• Journal of Chest Surgery
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• 제14권1호
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• pp.26-32
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• 1981

Three cases of tricuspid atresia were treated by Fontan varieties of operation in this department in 1980. The first case was 19 year old girl who underwent Glenn operation at the age of 6 years. Her second operation was done with ASD closure and 16mm Ionescu-Shiley valved conduit insertion between right atrium and main pulmonary artery. The second case was a 5 year old boy who underwent Kreutzer operation successfully utilizing 14mm Ionescu-Shiley va]ved conduit. The above mentioned 2 cases were Type Ib after Keith`s classification, whose immediate postoperative courses were complicated by pleura] effusion [in 2nd case chylothorax] hepatomegaly, and ascites. Those complications were relieved completely by medical treatment and closed thoractomy; Postoperative follow-up up to 11 months and 1 year periods were satisfactory with disappearance of cyanosis and dyspnea. The third case was a 8 year old boy who had complete TGA with TA [Keith`s Type IIb] who underwent Kreutze`s operation utilizing 14mm Ionescu-Shiley valved conduit, he died of low cardiac output Immediately after open heart surgery.

• Journal of Chest Surgery
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• 제20권1호
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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.

• 대한치과마취과학회지
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• 제12권1호
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• pp.45-50
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• 2012

The Fontan operation is a heart operation used to treat complex congenital heart defects like tricuspid atresia, hypoplastic left heart syndrome, pulmonary atresia and single ventricle. A single ventricle is dedicated to pumping oxygenated blood to the systemic circulation and the entire systemic venous return reaches the pulmonary arterial system without the direct influence of a pumping chamber. In the patient with Fontan operation, it is important to achieve adequate pulmonary blood flow and cardiac output in anesthetic management. In this case, a 10-year-old boy (19.6 kg, 114 cm) with cleft palate, cerebral palsy and severe mental retardation, who underwent a Fontan operation when he was 4 years old, was presented for deep sedation. Because he was suffering from eating disorder with cleft palate, the orthodontist and the plastic surgeon planned to insert intraoral orthodontic device before cleft palate repair. But it was impossible to open his mouth for alginate impression procedure. After careful pre-anesthesia evaluation we planned to administer deep sedation with propofol infusion. After Intravenous catheter insertion, we started propofol intravenous infusion with the formula of a loading dose of 1.0 mg/kg followed by an infusion rate of 6.0 mg/kg/hr with syringe pump. His blood pressure was remained around 80/40 mmHg after loss of consciousness, but he could not maintain his airway patent. So we lowered the infusion rate to 3.0 mg/kg/hr, immediately. The oxygen saturation was maintained above 95% with nasal oxygen supply, and blood pressure was maintained around 100-80/60-40 mmHg. After the sedation of 110 minutes with propofol (the infusion rate to 3.0-5.0 mg/kg/hr), he fully regained consciousness, and was discharged without complication after 1 hour observation. In case of post-Fontan patient, intravenous deep sedation with propofol was safe and effective method of behavioral management during dental treatment.

• Journal of Chest Surgery
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• 제34권1호
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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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• 제18권4호
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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.