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Prenatal detected Isolated Congenital Coronary Artery Fistula (ICCAF) characteristics and impact on the fetal hemodynamic situation: Systematic literature review

Adeline Walter, Elina Calite, Ulrike Herberg, Johannes Breuer, Christoph Berg, Annegret Geipel, Ulrich Gembruch

Background: Detection of an Isolated Congenital Coronary Artery Fistula (ICCAF) is rarely seen prenatally. We aimed to describe prenatal characteristic and try identify possible prognostic parameters that could guide prenatal assessment and improve counseling.

Methods and Findings: A systematic literature review was performed. PubMed database was reviewed for publications using the terms “isolated (congenital) coronary artery fistula, coronary-cameral fistula”. Papers with full description of the parental fistula characteristics were included for evaluation. 22 publications fulfilled search criteria describing a total of 25 pregnancies. Mean gestational age at diagnosis was 25.4 weeks of gestation (range: 19 and 38). ICCAF predominantly drained into the right side of the heart (81.5%) and in most cases into the right atrium in 52.4% (11/21). Prenatal sonographic findings included: Enlargement of the originated coronary artery in all cases (100%), enlargement of the draining structure in 8 (32%), cardiomegaly in 5 (20%), turbulent jet in 6 (24%), to-and-fro flow in the ascending aorta in 6 (24%) and a hydrops fetalis in 2 (8%), of the reviewed subjects. Prenatal findings of high volume shunting through the fistula, hydrops fetalis or aneurysm were associated with a more serious negative impact on fetal outcome.

Conclusion: Prenatal diagnosis of an ICCAF requires an examination of the affected fetal hemodynamic situation by the fistula. Significant derangements in cardiac function, may have relevant postpartale consequences. Evaluation of the coronary artery circulation and possible prognostic parameters as the presence of a cardiomegaly, isolated cardiac chamber enlargement, to-and-fro flow in the aortic arch, dilatation of one of the great vessels, the size, tortuosity, length of the fistula and hydrops fetalis should be examined and targeted management be performed.