This study describes the incidence and course of children with small coronary artery fistulae over a period of 6 years who presented at a paediatric tertiary-care centre.

Age at diagnosis, mode of presentation, location (origin and drainage), and association with a cardiac defect were documented and analysed. All patients obtained an electrocardiogram, and older patients were further evaluated with an exercise treadmill test.

A total of 31 patients were diagnosed with coronary artery fistula via transthoracic echocardiogram and comprised 0.43% of our entire patient group. Mean age was 6.14 years (standard deviation 5.4); 16 patients (52%) had associated cardiac defects. In the remaining 15 patients, the coronary artery fistula was discovered incidentally during diagnostic work-up for heart murmur or chest pain. Among all, 26 patients (84%) had left-sided and five patients (16%) had right-sided coronary artery fistulae. All right coronary artery fistula patients had associated cardiac defects; this was true for 42% of the patients with left coronary artery fistulae. None of the patients required any intervention due to the fistula, and spontaneous closure occurred in 12 patients (39%).

Small coronary artery fistulae in children are frequently an incidental finding, and many will close spontaneously. Our data are supportive of a conservative, observant approach in asymptomatic patients with small coronary artery fistula in the paediatric population.