Changes in pulmonary artery size during and after the staged extracardiac total cavopulmonary connection

Background: Optimal development of the pulmonary arteries (PAs) is crucial for Fontan circulation. This study investigated longitudinal changes in PA size in patients who underwent staged Fontan palliation and analyzed their impact on outcomes. Methods: Serial PA angiograms at bidirectional cavopulmonary shunt (BCPS), extracardiac total cavopulmonary connection (TCPC), and after TCPC were reviewed. PA index (PAI), right and left PAI, and PA symmetry index were calculated, and serial changes were compared. Results: = .006). Conclusions: During the staged Fontan procedure, the left PAI decreased, whereas the PAI and right PAI remained stable or increased. A PA symmetry index <0.61 predicts mortality following TCPC. This progressive asymmetry in left-to-right PA size affects in-hospital morbidity and emphasizes the importance of achieving balanced PA growth prior to Fontan completion.