Coronary arteriovenous fistula in pediatric patients: a 17-year institutional experience

Coronary arteriovenous fistula (CAVF) is a rare congenital anomaly in pediatric patients. Its clinical manifestations vary considerably and its long-term outcome is not fully understood. This study sought to determine the natural history and long-term outcome in CAVF patients treated over a 17-year period at Cathay General Hospital in Taipei.

The medical records of all 10 pediatric patients (five boys and five girls aged between 15 days and 15 years) with CAVF treated from 1983 through 2000 at our hospital were reviewed. Data collected included symptoms and signs, the findings of electrocardiography, echocardiography, catheterization, and angiography, and surgical results.

CAVF was diagnosed on the basis of color Doppler echocardiography in eight patients and by cardiac catheterization and angiography in two. Congestive heart failure was found in four patients and both myocardial ischemia and infarction were found in two patients. Most of the affected coronary arteries were tortuous and dilated with a mean diameter of 12.6 mm (range 5-40 mm). Under cardiopulmonary bypass, fistulous terminations were sutured in seven patients, three of whom were found to have multiple fistulous openings. Postoperative follow-up examinations revealed that all of the affected coronary arteries and fistulas remained dilated and tortuous, except in one patient. Two patients who had distal CAVF developed coronary thrombus, calcification, and ventricular aneurysm at 2 and 10 years after operation, respectively. Another patient developed fistulous recanalization 7 years after operation, but this abnormal channel had disappeared again 3 years after recanalization. One patient developed an iatrogenic CAVF 8 years after surgical repair of tetralogy of Fallot.

Unlike adults, pediatric patients with CAVF tend to be symptomatic. Ligation of the fistulous termination alone does not reduce the size of the fistula. Our findings indicate that long-term follow-up is essential due to the possibility of postoperative recanalization, persistent dilation of the coronary artery and ostium, thrombus formation, calcification, and myocardial infarction. In addition, postoperative antiplatelet therapy is recommended, especially in patients with distal CAVF and abnormally dilated coronary arteries.