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

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.