Coarctation of the aorta: difficulties in prenatal diagnosis.

OBJECTIVE To formulate echocardiographic criteria for the prenatal diagnosis of coarctation of the aorta. DESIGN A retrospective study examining the echocardiograms of fetuses with a verified aortic arch abnormality and those in whom the diagnosis was suspected prenatally but was not subsequently confirmed. SETTING Tertiary referral centre for fetal echocardiography. PATIENTS 87 fetuses in whom the diagnosis of coarctation was correctly made in 54, suspected but unproved in 24, and overlooked prenatally in nine. MAIN OUTCOME MEASURES Measurements of left and right ventricular size, the diameters of the great arteries, the diameters of the left and right atrioventricular valvar orifices, the appearance of the aortic arch, and the direction of the flow of blood across the foramen ovale. RESULTS Measurements of the ventricular widths, diameters of the great arteries, or the diameters of the atrioventricular valvar orifices, did not allow clear distinction between cases that definitely had a coarctation and those in whom the diagnosis was unproved. The appearance of the aortic arch, particularly in the horizontal projection, was more helpful in distinguishing cases of coarctation, although this also was not always diagnostic. A predominantly left to right shunt across the foramen ovale was detected more often in cases with a substantiated coarctation (58%) than in those with an unproved diagnosis (12%). CONCLUSIONS The most severe forms of coarctation are associated with relative hypoplasia of the left heart structures compared with the right and a correct diagnosis can be made in early pregnancy. The milder forms of coarctation, however, are consistent with a normal early fetal echocardiogram. In late pregnancy it may be impossible to exclude coarctation categorically as the right heart structures may appear larger than the left in the normal fetus. Thus although a combination of echocardiographic features can correctly identify aortic arch anomalies in the fetus, none either alone or in combination, could clearly distinguish between real and false positive cases, particularly in late gestation.