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Treatment of Pulmonary Atresia, Ventricular Septal Defect and Diminutive Pulmonary Arteries Comparing First Palliative Management Schemes

Journal of the Korean Pediatric Society 1999;42(6):800-806.
Published online June 15, 1999.
Treatment of Pulmonary Atresia, Ventricular Septal Defect and Diminutive Pulmonary Arteries Comparing First Palliative Management Schemes
Jean Yoon1, In Seung Park1, Soo Jin Kim1, Mi Young Han1, Do Jun Cho1, Eun Jung Bae1, Young Tak Lee2, Seong Ho Kim1
1Department of Pediatrics, Sejong General Hospital, Pucheon, Korea
2Department of Cardiovascular Surgery, Sejong General Hospital, Pucheon, Korea
극소 폐동맥을 갖는 심실 중격 결손을 동반한 폐동맥 폐쇄 환자의 초기 고식적 수술 방법에 따른 치료 성적
윤진1, 박인승1, 김수진1, 한미영1, 조도준1, 배은정1, 이영탁2, 김성호1
1부천세종병원 소아과
2부천세종병원 흉부외과
Correspondence: 
Seong Ho Kim, Email: 1
Abstract
Purpose
: This report reviews an 8-year treatment of pulmonary atresia, ventricular septal defect and diminutive pulmonary arteries, comparing first palliative management schemes.
Methods
: Between January 1989 and March 1997, patients had their pulmonary artery anatomy evaluated before any surgical managements. Twenty-two patients had diminutive pulmonary arteries(Nakata index<90). Clinical records, hemodynamic data, and cineangiograms were examined in these patients.
Results
: The median age of patients were 14 months and the mean Nakata index were 54.7±18.2(24.3-88.9). The cases were classified into 3 different groups according to different first palliative strategies. Group Ⅰ(n=18) was treated by a right ventricular outflow tract reconstruction. Group Ⅱ(n=2) was treated by unifocalization and Blalock-Taussig shunt, and Group Ⅲ(n=2) by a central shunt. The mean Nakata index of Group Ⅰ was 68.0±29.6 and Group Ⅱ and Ⅲ showed 71.9±13.1 and 41.0±13.1, respectively. The total correction was performed in 14 cases (77.8%) of Group Ⅰ and in 1 case (50%) of Group Ⅱ. Group Ⅰhad 3 deaths. Coil embolization was performed in 6 cases before total correction, and balloon angioplasty was performed in 3 and 5 cases, before and after the total correction, respectively. After total correction, the peak systolic pressure ratio of the right ventricle to the aorta was 0.80±0.08 in 11 cases. 5 cases of those indicated that balloon angioplasty reduced the ratio from 0.89 to 0.78.
Conclusion
: These results of first palliative surgery on the right ventricular outflow tract reconstruction compared favorably with previous reports of disease's history and survival after complete repair.
Key Words: Pulmonary atresia and Ventricular septal defect, Right ventricular outflow tract reconstruction, Interventional catheterization


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