Color Doppler of the splenic artery in the prenatal diagnosis of heterotaxic syndromes

The objective of this paper is to determine whether color and pulsed Doppler of the splenic artery is helpful in the prenatal diagnosis of polysplenia or asplenia in heterotaxic syndromes. Over a 3-year period, localization of the splenic artery by color and pulsed Doppler was attempted on all fetuses with the diagnosis of heterotaxic syndromes. Postnatal follow-up was obtained on all neonates. The diagnosis of heterotaxic syndromes was performed on eight fetuses during the study period. Mean gestational age at diagnosis was 20.1 weeks. All fetuses had situs ambiguous and complex cardiac abnormalities. All pregnancies were managed expectantly and none were terminated. The splenic artery was imaged by color and pulsed Doppler in 6 of 8 fetuses, all with one or multiple spleens confirmed postnatally. The splenic artery could not be imaged in two fetuses, both with asplenia confirmed postnatally. The perinatal mortality rate was 88% (7 of 8) and the one surviving infant is currently alive and well at 3 years of age. Color and pulsed Doppler of the splenic artery can aid in the prenatal diagnosis of heterotaxic syndromes. This information is of value and should result in improved prenatal counseling and management of affected pregnancies.