• Journal of Chest Surgery
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• 제22권2호
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.

• Journal of Chest Surgery
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• 제49권5호
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• pp.374-378
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• 2016

A multistage plan and multidisciplinary approach are the keys to successful repair in patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). In this article, we present a multidisciplinary approach adopted to treat a patient with PA with VSD and MAPCAs associated with left pulmonary artery interruption.

• Journal of Chest Surgery
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• 제20권2호
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• pp.404-410
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• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Journal of Chest Surgery
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• 제54권5호
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• pp.389-392
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• 2021

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

• Journal of Chest Surgery
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• 제25권11호
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• pp.1327-1336
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• 1992

Between Jan. 1986 and Aug. 1992. 72 patients underwent Rastelli procedure. There were 43 male and 29 female, aged 46 days to 16 years [mean age, 5.2 years] with 18 patients less than 2 years of age. All patients had complex defect, 27 pulmonary atresia with ventricular septal defect, 18 corrected transposition of great arteries with pulmonary atresia or punmonary stenosis, 10 truncus arteriosus, 10 double outlet right ventricle with pulmonary atresia or stenosis, 7 complete transposition of great artersia with pulmonary atresia or pulmonary stenosis. The types of extracardiac valved conduit used were prosthetic valve[n=47, 24 car-bomedics, 19 Ionescu-Shiley, 4 Bjork-shiley] and hand-made trileaflet valve using pericardium. [n=23, 20 bovine pericardium, Z autologous pericardium, 1 equine pericardium] The mean size of valved cinduit was 5.25mm larger in diameter than the size of main pulmonary artery. [normalized to the patient`s body surface area] There were 17 hospital death[24%] and 4 late deaths[5.6%]. Postoperative complication rate was 38.9%a, none of which was conduit-related. All patients were followed pos-toperatively for 1 to 73 months. [mean 25.8 months] During follow-up period, reoperation was done in 6 patients due to stenosis of valved conduit. Mean interval between intial repair and reoperation was 20.3 months. In our experience, li recently extracardaic valved conduits between right ventricle [or pulmonary ventricle] and pulmonary artery were inserted with increasing frequency in infants less than 2 year, but hospital mortality was decreased, 2] Risk of reoperation due to conduit stenosis is low, so that the effect of graft failure on overall survival is minimized. 3] Nevertheless, because any type of extracardaic valved conduit is not ideal in children, we recommended that Lecompte should be done if cardiac anatomy is permitted.

• Clinical and Experimental Pediatrics
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• 제48권11호
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• pp.1256-1256
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• 2005

심실중격결손을 동반하지 않은 폐동맥 폐쇄(pulmonary atresia with intact ventricular septum)는 전체 선천적 심장병의 1%를 차지하는 드문 질환으로 형태학적 특성에 따라 다양한 치료적 접근이 필요하다. 이에 저자들은 정상 크기발달의 우심실을 가진 심실중격결손을 동반하지 않은 폐동맥 폐쇄가 있는 신생아에서 풍선판막성형술 후 지속적인 저산소증을 보여 동맥관 스텐트를 이용하여 거의 정상적인 심장구조와 기능으로 호전된 1례를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제32권9호
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• pp.823-826
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• 1999

주대동맥폐동맥간 부행혈관, 심실중격결손과 폐동맥 형성부전이 동반된 6개월된 환아에서 Clamshell 절개 를 통한 일차 완전교정술을 시행하였다. 수술 후 특별한 합병증은 없었으며, 초음파 검사상 남아있는 심실중 격결손이나 의미있는 폐동맥 협착 소견을 보이지 않았다. 이러한 질환의 수술적 접근에 \ulcorner폐로 공급되는 혈 류의 형태에 따라 다양한 방법이 존재하는데, 최근 들어서는 일차 완전교정술에 대한 여러 형태의 수술방법 이 제시되고 있으며, 그 적용 또한 확대되고 있다. 본 증례의 경우 Clamshell 절개 방법을 적용함으로써 종격 동내 병변에 대한 접근이 용이하였으며, 수술 결과 및 합병증 등의 측면에서도 일차 완전교정술의 한 방법 으로 고려해 볼만하다고 여겨진다.

• Journal of Chest Surgery
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• 제30권3호
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• pp.322-325
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• 1997

세종병원 흉부외과에서는 폐동맥 페색증, 좌측폐동맥 무형성증, 심실중격결손증, 다발성 MAPCA등을 동반한 상행 대동맥으로부터 이상기시한 우폐동맥 11례를 경험하였다. 10개월 때 1단계 수술로써 좌측 unifocalization과 함께 좌폐동맥을 만들고 우폐동맥 벤딩을 하였고, 1개월후 우측 MAPCA의 coil 색전술 단계를 거쳐, 1년후 완전 교정술을 시행하였다. 술후 경과는 양호하였고 수술 1년후 조영술과 혈역학적 검사상 만족스런 결과를 얻을 수 있었다.

• Journal of Chest Surgery
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• 제24권2호
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• pp.212-216
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• 1991

A surgical correction was successfully performed in a adult female who had pulmonary atresia with almost non-confluent pulmonary artery, PDA and ventricular septal defect. Initially as a first stage of corrective surgery isolation of patent ductus arteriosus, ligation of aortopulmonary collaterals and identification of the pathologic anatomy of left pulmonary artery through left posterolateral approach were performed. At the second stage, a week after the initial operation, total correction was done making both pulmonary arteries confluent with albumin coated woven-dacron graft, external valved conduit and closure of large VSD of subarterial and perimembranous type. The PDA was ligated with previously encircled heavy stitch soon after partial bypass was started. Although massive bleeding from anastomotic site of dilated left pulmonary artery to the graft occurred preoperatively, postoperative functional improvement was excellent in terms of disappearance of cyanosis and normal exercise tolerance.

• Journal of Chest Surgery
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• 제46권1호
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• pp.56-59
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• 2013

Cardiac surgery in neonates with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight neonates with congenital heart disease continue to challenge the intellectual and technical skills of healthcare professionals. We present a case of a low-birth-weight neonate with pulmonary atresia and a ventricular septal defect, in whom palliation was achieved with a right ventricular outflow tract stent using a hybrid procedure.