• Journal of Chest Surgery
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• v.22 no.2
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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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• v.49 no.5
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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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• v.20 no.2
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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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• v.54 no.5
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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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• v.25 no.11
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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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• v.48 no.11
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• pp.1256-1256
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• 2005

Pulmonary atresia with intact ventricular septum (PAIVS) is rare, less than 1% of congenital heart disease. It needs a therapeutic approach according to its individual morphologic feature. Surgical treatment of valvotomy and modified Blalock-Taussig shunt or non-surgical interventional catheter balloon valvuloplasty can be used for mild to moderate hypoplasia of right ventricle. Fontan operation can be considered for less optimum morphological substrate of two ventricular repair. A 3-day-old male neonate was admitted with cyanosis and cardiac murmur. On echocardiogram, he had membranous pulmonary atresia with intact ventricular septum, normal sized tripartite right ventricle, large atrial septal defect with right-to-left shunt, small sized patent ductus arteriosus, and moderate tricuspid regurgitation. He was treated with intravenous continuous infusion of prostaglandin $E_1$ ($PGE_1$) at once. On the third day of hospitalization, Balloon valvuloplasty was performed. After insertion of patent ductus arteriosus stent on the tenth day, $PGE_1$ infusion was discontinued. On the fifteenth day, he was discharged. Now, he is 9 months old and has nearly normal cardiac structure and function with 97% of percutaneous oxygen saturation.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.823-826
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• 1999

A 6-month old girl who had pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals underwent one-stage complete repair with unifocalization through a bilateral thoracosternotomy(clamshell incision). There were no serious postoperative compli cations, and the postoperative echocardio-graphy showed no residual ventricular septal defect or significant pulmonary artery stenosis. In this condition, great surgical variability exists regarding the sources of pulmonary blood flow. Recent clinical work has focused on a one-stage complete repair. The potential advantages of the clamshell incision are apparent in terms of mediastinal approach, postoperative results, and safety.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.322-325
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• 1997

We experienced a case of anomalous origin of right pulmonary artery from the ascending aorta associated with pulmonary atresia, ventricular septal defect, absence of left pulmonary artery afld multiple major aortopulmonary collateral artery (MAPCA). At ten month of age, left pulmonary artery creation with unifocalization and right pulmonary artery banding were performed as the Urst stage, followed by coil embolization of right MAPCA 1 month later, and 1 year later, the total correction was done. After total repair, the patient showed good postoperative course and excellent angiographic and hemodynamic results at 1 year follow-up study.

• Journal of Chest Surgery
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• v.24 no.2
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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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• v.46 no.1
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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.