All issues > Volume 42(6); 1999

Original Article

J Korean Pediatr Soc. 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 J Yoon¹, In Seung IS Park¹, Soo Jin SJ Kim¹, Mi Young MY Han¹, Do Jun DJ Cho¹, Eun Jung EJ Bae¹, Young Tak YT Lee², Seong Ho SH Kim¹

¹Department of Pediatrics, Sejong General Hospital, Pucheon, Korea
²Department of Cardiovascular Surgery, Sejong General Hospital, Pucheon, Korea

Correspondence Seong Ho SH 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.

Keywords :Pulmonary atresia and Ventricular septal defect, Right ventricular outflow tract reconstruction, Interventional catheterization