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Pulmonary artery growth fails to match the increase in body surface area after the Fontan operation

Objective: To evaluate the growth of the pulmonary arteries after a Fontan procedure. Design: Retrospective review. Setting: Two paediatric cardiology tertiary care centres. Patients: 61 children who underwent a modified Fontan operation and had angiography suitable for assessment of pulmonary artery size before the Fontan procedure and during long term follow up. An atriopulmonary connection (APC) was present in 23 patients (37.7%) and a total cavopulmonary connection (TCPC) was present in 38 (62.3%). Postoperative angiograms were performed 0.5–121 months (median 19 months) after the Fontan operation. Main outcome measure: Growth of each pulmonary artery measured just before the first branching point. The diameter was expressed as a z score with established nomograms used to standardise for body surface area. Results: The mean change in the preoperative to postoperative z scores of the right pulmonary artery was −1.06 (p  =  0.004). The mean change in the preoperative to postoperative z scores of the left pulmonary artery was −0.88 (p  =  0.003). Changes in the preoperative to postoperative z scores were more pronounced in the patients undergoing APC than TCPC, especially for the right pulmonary artery. Conclusion: After the Fontan operation, growth of the pulmonary arteries often fails to match the increase in body surface area.