Pediatric Interventions: Timing is Everything

by Yimin Hua, MD

Coronary artery fistula is considered a major coronary anomaly that is present in 0.002% of the general population and accounts for 0.4% of all cardiac malformations. Most children with CAF are asymptomatic. It is still controversial about the best management of asymptomatic CAF, since both spontaneous closure and life-threatening complications have been reported. Prior to this study, there is a paucity of information regarding the prognostic implications of incidentally identified, clinically silent CAF. Additionally, regarding the efficacy and safety of transcatheter closure of CAFs, many studies conducted in adult patients have been published in the literature. However, complications including device migration, recanalization of the fistula, coronary thrombus formation, and myocardial ischemia or infarction have been reported. While the data from adult patients may serve as good reference for child patients, the outcome of CAF closure in child patients should be carefully evaluated as a separated group, because of their growing nature and according anatomic changes. However, very limited information is available for the intermediate and long-term outcomes after transcatheter closure in children. With the absence of data regarding follow-up outcomes, it is difficult to make recommendations for management strategies and the optimal timing for the intervention.
Encouragingly, we confirmed that the greater initial size of maximal coronary artery diameter (MCD) and/or fistulous orifice diameter (FOD) in asymptomatic children with CAF, the more possible it is related to the late occurrence of complications (gradually increased MCD and FOD, aneurysm formation, and symptoms of heart failure). Based on our experience, we recommend the closure of asymptomatic CAF in children as early as possible if MCD ≥5 mm and/or FOD >2 mm. In addition, based on the favorable intermediate-term follow-up outcome, we think that transcatheter closure of CAF with a patent ductus arteriosus (PDA) occluder is an effective and safe approach in appropriately selected children with moderate and large CAF. Furthermore, we believe that the closure of moderate and large distal CAFs at a younger age may provide a favorable condition for remodeling conduit coronary artery and reducing risk for thrombosis.
In the mean time, our study has some limitations, including the small sample size and unavailability of follow-up angiogram or MDCT in all patients. In addition, it is necessary to optimize the duration for antiplatelet/anticoagulation following CAF closure. Furthermore, management of CAF in the current study was primarily based on expert consensus as opposed to a large randomized controlled trial. Further investigation is still necessary to make definitive recommendations. Lastly, some severe complications like myocardial infarction may occur much later after CAF closure. Therefore, multicenter longitudinal studies with more patients and long-term follow-up are still needed to better understand the best management approach for this rare but important disorder in children.

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A selection of articles on Coronary Artery Fistulas:

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