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OBJECTIVE: Pulmonary atresia with intact ventricular septum is a complex congenital cardiovascular anomaly that frequently requires single ventricle palliation. Fetal diagnosis of pulmonary atresia with intact ventricular septum is common, but the natural history of pulmonary atresia with intact ventricular septum diagnosed in midgestation, predictors of neonatal anatomy, and predictors of biventricular repair have not been determined. The objective of this study was to determine whether the size and rate of growth of the fetal tricuspid valve predict neonatal anatomy and biventricular repair. DESIGN AND RESULTS: Twenty-three fetuses diagnosed with pulmonary atresia with intact ventricular septum between 1990 and 2004 were studied. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 1 achieved biventricular repair, compared with 5 of 5 with a tricuspid valve z score >--3. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 8 were diagnosed postnatally with a right ventricular dependent coronary circulation, compared with none with a tricuspid valve z score >--3. Midgestation and late gestation fetal tricuspid valve z scores correlated with neonatal tricuspid valve z score. The average rate of tricuspid valve growth between mid- and late fetal echocardiograms was significantly lower in patients who did not achieve biventricular repair than in those who did (0.012 +/- 0.008 cm per week vs 0.028 +/- 0.014 cm per week). CONCLUSIONS: Fetal tricuspid valve z score and rate of growth predict postnatal outcome in pulmonary atresia with intact ventricular septum. These findings may have important implications for prenatal counseling and selection of patients for fetal pulmonary valve dilation. [ABSTRACT FROM AUTHOR]