The objective of this study is to evaluate the efficacy of sonographic card
iothoracic ratio at midpregnancy in predicting fetal hemoglobin Bart diseas
e. Among 17,254 pregnant women screened for severe thalassemia between Tune
1994 and November 1998, 345 pregnant women at risk for having a fetus with
hemoglobin Bart disease underwent ultrasonographic examination and cordoce
ntesis at 18 to 21 weeks' gestation. Before cordocentesis, the cardiothorac
ic ratio was determined and recorded. The definite fetal diagnosis was base
d on fetal blood analysis with high performance liquid chromatography. Amon
g 345 pregnancies in which sonographic examination and cordocentesis were p
erformed, 70 fetuses were affected by hemoglobin Bart disease. The mean car
diothoracic ratio was significantly higher than that of unaffected fetuses
(0.55 versus 0.45, Student's t-test, P < 0.001). The sensitivity and specif
icity of the cardiothoracic ratio in prediction were calculated for various
cutoff values. On the basis of the receiver operating characteristic curve
, the best cutoff value was 0.50 (greater than 0.50 considered abnormal), g
iving the sensitivity of 98.6% and a specificity of 98.9%. In conclusion, t
he cardiothoracic ratio has very high accuracy in predicting hemoglobin Bar
t disease in pregnancies at risk. This finding suggests that invasive diagn
ostic method should be reserved for only the fetuses who have a cardiothora
cic ratio of 0.5 or more; however, further studies are needed to confirm th
is observation.