The aim of this study was to describe the clinical characteristics, progression, treatment, and outcomes in isolated coronary artery fistula cases diagnosed prenatally.

We carried out a retrospective review of babies diagnosed prenatally with coronary artery fistulas between January, 2000 and December, 2013; five fetuses were included. Echocardiographic features and measurements were noted during pregnancy and after birth. Treatment and outcome were noted.

Gestational age at initial diagnosis was between 19 and 22 weeks; four coronary artery fistulas originated from the right and one from the left circumflex coronary artery. Drainage was into the right atrium in four cases and into the left ventricle in one case. There was cardiomegaly in two cases at the initial scan. The size of the fistulas increased during pregnancy in all except one. All prenatal diagnoses were confirmed postnatally. Among all, two patients developed congestive cardiac failure soon after birth and required transcatheter closure of the coronary artery fistula, 5 and 17 days after birth, respectively; three patients remained asymptomatic, and all of them showed progressive dilation of the feeding artery and had closure of the fistula at 20 months, 4 years, and 7 years of age, respectively. During the follow-up period, which ranged 2–14 years, all the patients were alive and well.

Coronary artery fistulas can be diagnosed accurately during fetal life. Some babies may develop congestive cardiac failure soon after birth requiring early treatment. Those treated conservatively should be kept under review as intervention may be required later.