Background Cardiac anomalies may be associated with abnormal coronary vascu
lar connections. We report the prenatal diagnosis of ventriculocoronary fis
tula in three fetuses with associated cardiac anomalies. Materials and
Methods Fetal echocardiography was performed in three patients referred for
suspected cardiac anomaly. Two-dimensional fetal echocardiography was comp
lemented by color Doppler flow imaging and spectral Doppler in all cases.
Results A ventriculocoronary, fistula was diagnosed in three patients refer
red at 22, 23 and 32 weeks. The first patient had hypoplastic left heart as
sociated with transposition of the great arteries and pulmonary atresia wit
h an intact interventricular septum. The coronary fistula arose from the tr
ansposed aorta to the left ventricle. In two patients ventriculocoronary fi
stula was found in association with pulmonary, atresia and an intact interv
entricular septum. hr all cases there was bidirectional flow within the fis
tula (diastolic blood flow towards the ventricle with reversal during ventr
icular systole). The pregnancy with hypoplastic left heart with transpositi
on, and one of those with pulmonary atresia resulted in neonatal death and
stillbirth, respectively. In the third instance the ventriculocoronary fist
ula was verified by postpartum cardiac angiography. The infant initially re
ceived a Blalock-Taussig shunt, subsequently replaced by a bidirectional Gl
enn shunt, and was doing well at the time of writing.
Conclusion A ventriculocoronary fistula can be identified prenatally by col
or and spectral Doppler. This anomaly should be sought in fetuses with outf
low tract obstructive cardiac lesions and an intact interventricular septum
. Prenatal diagnosis allows early angiography postnatally. Delineation of c
oronary, vascular regions may therefore facilitate preoperative planning.