DEVELOPMENT OF PULMONARY ARTERIOVENOUS-FISTULAS IN CHILDREN AFTER CAVOPULMONARY SHUNT

Background The cavopulmonary shunt procedure is now used for palliatio n of complex congenital heart lesions in infants. While pulmonary arte riovenous fistulae (PAVF) are a well-known complication of this surger y in older patients, no study of the prevalence of this condition in c hildren and young infants has been reported. Methods and Results We co mpared 29 patients with cavopulmonary shunts or total caval exclusion with 53 control subjects evaluated by contrast echocardiography at the University of California, San Francisco. The primary cardiac lesion, age at the time of surgery, type of right heart bypass procedure, prov ision of auxiliary pulmonary blood flow, and changes in oxygen saturat ion over time were compared. The prevalence of PAVF in children after cavopulmonary anastomosis is 60%, higher than previously reported. The prevalence is significantly higher in infants <6 months old and in th ose with a heterotaxy syndrome. The provision of an additional source of pulsatile, pulmonary blood flow appears to have little effect on th e development of PAVF. Patients who developed PAVF had arterial oxygen saturations at the time of discharge from surgery similar to those wh o did not develop them. Those with PAVF had significantly lower arteri al and pulmonary venous oxygen saturations at follow-up as a result of their intrapulmonary shunt. Conclusions Contrast echocardiography pro vides a sensitive method for the detection of PAVF. While the origins, natural history, and ultimate clinical significance of PAVF in childr en after cavopulmonary anastomosis are unclear, surveillance by contra st echocardiography is indicated for all patients who have had this pr ocedure because PAVF may cause significant intrapulmonary right-to-lef t shunting in some patients.