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

Abstract

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
• prenatal diagnosis
• perinatal prognosis

Acknowledgments

We sincerely acknowledge all the co-authors who have collaborated in the development of this study and we are grateful to the mother who has selflessly contributed to the study.

Authors’ contributions

All authors have accepted responsibility for the entire content of this manuscript and approved its submission. All authors read and approved the final manuscript.

Ethical approval

The study was conducted in line with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines (11) and approved by the Institutional Review Board of the Hospital Universitario 12 de Octubre Research Institute (approval number 23/118). It was conducted ethically in accordance with the World Medical Association Declaration of Helsinki, and informed consent was obtained from the patient from the included case report from our center.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Data availability statement

The datasets used and/or analyses performed during the study are available from the corresponding author upon reasonable request.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.