Reversed shunting across the ductus arteriosus or atrial septum in utero heralds severe congenital heart disease.

OBJECTIVES: This study was performed to define the significance of Doppler color flow mapping in demonstrating reversal of the direction of the normal physiologic flow across the atrial septum and ductus arteriosus in the human fetus.

BACKGROUND: Reversal of the physiologic shunting across the ductus arteriosus or atrial septum in utero (i.e., left to right) can be readily identified by Doppler color flow mapping, complemented by pulsed and continuous wave Doppler information.

METHODS: We reviewed echocardiograms recorded at our three institutions from 1988 to 1993, which displayed reversal of flow by Doppler color flow in 53 fetuses of gestational age 18 weeks to term. The diagnoses were confirmed by postnatal echocardiography, operation or autopsy. Reversal of shunting was consistently associated with severe heart disease.

RESULTS: Reversed atrial shunting was found with severe left heart obstructive lesions, including 19 with hypoplastic left heart syndrome, 3 with critical aortic stenosis, 2 with double-outlet right ventricle and 1 each with an interrupted aortic arch, atrioventricular septal defect and severe left ventricular dysfunction due to dilated cardiomyopathy. Reversed ductus arteriosus shunting was found with severe right heart obstructive lesions, including nine fetuses with pulmonary atresia, six with severe obstructive tricuspid valve abnormalities, five with severe tetralogy of Fallot, four with Ebstein's anomaly and two with single ventricle and pulmonary stenosis. Associated cardiac lesions were common in both groups. Only 3 of the 15 infants who were delivered alive from the reverse ductus arteriosus shunt group and 4 of 12 from the reverse atrial shunt group still survive.

CONCLUSIONS: The finding of reversed flow by Doppler color flow mapping during fetal life provides a key to subsequent accurate diagnosis and denotes a spectrum of diseases with a very poor prognosis.