Prenatal diagnosis of isolated coronary artery fistula: systematic review, analysis of perinatal prognostic factors and case report

J Matern Fetal Neonatal Med. 2023 Dec;36(1):2206938. doi: 10.1080/14767058.2023.2206938.


Background: Isolated coronary artery fistula (CAF) is a rare entity in which evidence for both prognosis and need for perinatal treatment is lacking. We aim to evaluate the characteristics, evolution and perinatal outcomes of reported cases, including one from our center.

Material and methods: We performed a systematic review in Medline, Pubmed, and Embase databases for cohort studies or case series related to prenatally diagnosed isolated congenital CAF according to PRISMA guidelines. The search was restricted to articles published until January 2022, including a case report from our center. A descriptive analysis was performed, and perinatal characteristics were dichotomized by outcome (development of symptoms, as well as the need for surgery during the neonatal period). Strength of association between prenatal variables and outcome was evaluated through Odds Ratio.

Results: Only 27 cases of prenatal diagnosis of isolated CAF have been published, including our patient. Most had their origin in the right coronary artery (63%) and drained in the right ventricle (55.6%). Most cases (72%) developed progressive intrauterine dilation of the fistulous tract, which was usually associated with symptoms of cardiac overload, such as cardiomegaly (57.7%). Up to two-thirds of prenatally diagnosed patients developed heart failure symptoms in the neonatal period, and 84% required postnatal intervention. Prenatal diagnosis of both cardiomegaly and diastolic steal is associated with an OR of 52 and 41 of developing postnatal symptoms.

Conclusion: Prenatal diagnosis of isolated CAF can be achieved with adequate tools and trained sonographers. The development of cardiomegaly and diastolic steal significantly increases the risk of developing postnatal symptoms.

Keywords: Coronary artery fistula; congenital heart disease; echocardiography; perinatal prognosis; prenatal diagnosis.

Publication types

  • Systematic Review
  • Case Reports

MeSH terms

  • Cardiomegaly
  • Coronary Vessels / diagnostic imaging
  • Female
  • Fistula*
  • Heart Defects, Congenital*
  • Humans
  • Infant, Newborn
  • Pregnancy
  • Prenatal Diagnosis
  • Prognosis
  • Vascular Diseases*