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Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children

  • Congenital Heart Disease (RA Krasuski and G Fleming, Section Editors)
  • Published:
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Abstract

Purpose of Review

Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described. This review presents the classifications of CAFs in children and the short and long-term outcomes of CAF closure in children in the reported literature. We also summarize previously-reported angiographic findings and post-treatment remodeling characteristics in pediatric patients.

Recent Findings

With advancements in cross-sectional imaging technologies, anatomic delineation of CAFs via these modalities has become crucial in procedural planning. Recent reports of surgical and transcatheter closure of CAFs in children have reported good procedural success and low rates of short-term morbidity and mortality. Distal-type CAFs have elevated risk for long-term sequelae post-closure compared to proximal-type CAFs. A recent report of a multi-institutional cohort also describes post-closure remodeling classifications which may predict long-term outcomes in these patients as well as guide individualized anticoagulation management.

Summary

Invasive closure of significant CAFs via surgical or transcatheter techniques is feasible and safe in most children with good short and intermediate-term outcomes. However, close clinical and imaging follow-up is required to monitor for late complications even after successful closure. Antiplatelet and anticoagulation regimens remain important aspects of post-closure management, but the necessary intensity and duration of such therapy remains unknown.

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Authors and Affiliations

  1. Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Children’s Health System of Texas, Childrens Medical Center, 1935 Medical District Drive, Dallas, TX, 75235, USA

    Stephan Wu, Munes Fares, Thomas M. Zellers, Maanya Jyothinagaram & Surendranath R. Veeram Reddy

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  1. Stephan Wu
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Contributions

S.W., M.F., T.Z, and SR wrote the main manuscript text. S.W prepared Figs. 2, 3 and 7, S.R. prepared Figs. 4, 5 and 8, M.J. prepared Figs. 1, 6, and 9. All authors reviewed the manuscript.

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Correspondence to Stephan Wu.

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Wu, S., Fares, M., Zellers, T.M. et al. Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children. Curr Cardiol Rep 25, 1921–1932 (2023). https://doi.org/10.1007/s11886-023-02007-0

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