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

Yoon, Jean; Park, In Seung; Kim, Soo Jin; Han, Mi Young; Cho, Do Jun; Bae, Eun Jung; Lee, Young Tak; Kim, Seong Ho

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.