• Journal of Chest Surgery
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• v.28 no.11
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• pp.1045-1048
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• 1995

Pulmonary atresia with VSD is uncommon congenital anomaly with high mortality in neonatal period.Recently we experienced surgical correction of pulmonary atresia with VSD. The case was 2 month old male patient diagnosed as pulmonary atresia with VSD and PDA. Atretic pulmonary artery segment from Rt ventricular infundibulum to pulmonary artery was lcm in length. The pulmonary trunk tapered toward Right ventricular infundibulum and resulted in blind pouch with diameter of lmm. The left pulmonary artery was stenosed at just proximal and distal part to which PDA was connected. Total correction was undertaken which consisted of PDA ligation, dacron patch closure of VSD, establishment of continuity between right ventricle and pulmonary artery with autogenous pericardium. Postoperative systolic fight ventricular pressure and left ventricular pressure ratio was 0.7. In patient with pulmonary atresia with VSD it is advisable to perform a corrective operation, whenever the size and anatomy of pulmonary artery are acceptable for it.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.780-785
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• 1987

Pulmonary atresia with VSD is uncommon congenital anomaly with high mortality in neonatal period. Recently we experienced surgical correction of 2 cases of pulmonary atresia with VSD. The first case was 7-year old female patient and diagnosed as pulmonary atresia with VSD combined PDA. So, total correction was undertaken which consisted of PDA ligation, patch repair of VSD, transannular enlargement of RVOT with woven Dacron vascular graft, and closure of PFO. Postoperative systemic Rt. ventricular and radial artery pressure ratio was 0.44 and her postoperative course was uneventful. The second case was 6-year old male patient diagnosed as pulmonary atresia with VSD and large systemic-pulmonary collateral arteries. There were two large systemic-pulmonary collaterals, one was simply controlled by ligation, but the other was considered to supply Rt. upper lung. So end to side anastomosis was performed to the RVOT patch. Postoperative systolic Rt. ventricular and radial artery pressure ratio was 0.54. During the follow up period he showed clinical picture of Rt. heart failure, which is relatively well controlled with anticongestive therapy.

• Journal of Chest Surgery
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• v.19 no.3
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• pp.464-469
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• 1986

Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.

• Journal of Chest Surgery
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• v.25 no.1
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• pp.23-31
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• 1992

The ideal approach in the staged management of patients with pulmonary atresia has been a challenging problem and the result has not been always satisfactory. We reviewed our early result of initial palliative surgeries in fifteen cases of pulmonary atresia with interventricular communication Included are eight cases of simple pulmonary atresia with ventricular septal defect and seven cases of pulmonary atresia associated with other complex cardiac anomalies. The ages of the patients were less than one year except one. The morphology of pulmonary vasculature was highly variable and showed unfavorable conditions in most cases. Pulmonary artery was nonconfluent in two. Two-thirds of all cases showed significant problems such as juxtaductal stenosis or diffuse hypoplasia. The ductus arteriosus usually narrowed at its pulmonic end. Initial palliation was done by modified Blalock-Taussig shunt in six, central shunt with or without pulmonary angioplasty in five, right ventricular outflow tract [RVOT] reconstruction in three and direct connection of nonconfluent pulmonary arteries with bilateral cav-opulmonary shunt in one patient. There were 3 hospital deaths. Two of them underwent simultaneous repair of the associated anomaly of TAPVR. Among the six patients who received modified Blalock-Taussig shunt, three needed early second palliative procedure by central shunt, RVOT patch reconstruction and pulmonary angioplasty in each case, All patients who received central shunt showed marked clinical improvement. Among the twelve patients who survived the palliative procedures, two patients underwent total correction 13 months and 18 months after initial palliation respectively. We think that the choice of palliative procedure must be individualized according to the morphology of the pulmonary arteries. More experience and long term follow-up data are necessary to meet this challenging problem.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1212-1218
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• 2005

Purpose : Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. Methods : Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. Results : Ten(67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. $O_2$ saturation and pH were lower and cardiothoracic(CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia(P<0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but $O_2$ saturation was lower in functional atresia group(P<0.05). 13 patients(87%) were managed with $PGE_1$. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period(mean 37 months). 3 patients(20%) died and none of patients without pulmonary atresia died. Conclusion : We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.

• Journal of Chest Surgery
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• v.20 no.2
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• pp.432-437
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• 1987

Pulmonary atresia with intact ventricular septum is a extremely rare congenital cardiac anomaly. With the history of cyanosis and failure to thrive, this anomaly should not be excluded and emergency management is necessary. Our patient was 69 day-old male with pulmonary atresia which was confirmed by cardiac angiography. Prostaglandin E1 was used for maintenance of pulmonary blood flow preoperatively. Right ventricular outflow reconstruction with pericardial patch and concomitant pulmonary valvotomy were done on beating heart for palliation. With this method, growing of right ventricle and tricuspid annulus are highly expected.

• Journal of Chest Surgery
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• v.24 no.7
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• pp.719-724
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• 1991

A case of pulmonary atresia with intact ventricular septum was presented in a 10-month-old cyanotic female patient, which was congenitally rare. Infant with pulmonary atresia and intact ventricular septum usually require urgent surgical intervention. Angiogram showed the pulmonary atresia at the level of the pulmonary valve, the hypoplasia of tricuspid valve and atrial septal defect without patent ductus arteriosus. We performed the pericardial patch graft on the right ventricular outflow tract and pulmonary artery after ventriculotomy using pacemaker wire as electrical saw and main pulmonary arteriotomy and then modified Waterston shunt from the ascending aorta to patch on the right ventricular outflow tract without extracorporeal circulation. Patient was postoperatively good condition.

• Journal of Chest Surgery
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• v.22 no.5
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• pp.766-774
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• 1989

Four neonates with critical pulmonary outflow obstruction underwent emergency palliative operation between February 1988 and May 1989 at the department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital. There were three boys and one girl, the mean age was 12.25 days [ranging from 3 days to 26 days], and the mean body weight was 3,625 gm [ranging from 3,450 gm to 4,200 gm]. Two patients had severe pulmonic valvular stenosis with intact ventricular septum, a third had pulmonary atresia with intact ventricular septum, and another had pulmonary atresia with ventricular septal defect. All were cyanotic, showed arterial desaturation with metabolic acidosis, and had congestive heart failure. To relieve the pulmonary outflow tract obstruction, we performed transventricular pulmonary valvulotomy [Brock operation] with a mosquito clamp in all cases without extracorporeal circulation. Three patients survived the operation and showed satisfactory postoperative results. The patient who had pulmonary atresia with ventricular septal defect expired 5 days after surgery. We consider transventricular pulmonary valvulotomy [Brock operation] with a mosquito clamp as one of the preferable procedures for critical pulmonary outflow obstruction in the neonatal period.

• Journal of Chest Surgery
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• v.15 no.4
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• pp.394-401
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• 1982

Recently, 4 cases underwent successful surgical correction of tetralogy of Fallot combined with pulmonary atresia in 2 cases, with abnormal coronary artery in another 2 cases. The operation consisted of a patch repair to the ventricular septal defect. The pulmonary atresia and stenosis were corrected with the method of external connection, from the right ventricle to the pulmonary artery using the valved conduits.

• Journal of Chest Surgery
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• v.30 no.5
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• pp.524-527
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• 1997

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a rare and complex lesion in which great morphologic variability exists regarding the sources of pulmonary blood flow. We report a case of this disease with no true central pulmonary arteries in a 9-month-old-boy successfully treated by one-stage complete unifocalization and repair from a midline stemotomy approach.