Impacts of stage 1 palliation and pre-Glenn pulmonary artery pressure on long-term outcomes after Fontan operation

OBJECTIVES: The present study was aiming to determine whether high mean pulmonary artery pressure before bidirectional cavopulmonary shunt is a risk factor for late adverse events in patients with low pulmonary artery pressure before total cavopulmonary connection (TCPC). METHODS: We retrospectively reviewed the medical records of all patients undergoing both bidirectional cavopulmonary shunt and TCPC with available cardiac catheterization data. RESULTS: A total of 316 patients were included in this study. The patients were divided into 4 groups according to mean pulmonary pressure: those with pre-Glenn <16 mmHg and pre-Fontan <10 mmHg (Group LL, n = 124), those with pre-Glenn ≥16 mmHg and pre-Fontan <10 mmHg (Group HL, n = 61), those with pre-Glenn <16 mmHg and pre-Fontan ≥10 mmHg (Group LH, n = 66) and those with pre-Glenn ≥16 mmHg and pre-Fontan ≥10 mmHg (Group HH, n = 65). Group HL showed significantly higher rate of adverse events after TCPC than Group LL (P = 0.02). In univariate linear analysis, a history of atrial septectomy at stage 1 palliation was associated with low pre-Glenn mean pulmonary artery pressure (Coefficient B-1.38, 95% confidence interval-2.53 to-0.24; P = 0.02), while pulmonary artery banding was a significant risk factor for elevated pre-Fontan mean pulmonary artery pressure (Coefficient B 1.68, 95% confidence interval 0.81 to 2.56, P < 0.001). CONCLUSIONS: High mean pulmonary artery pressure before bidirectional cavopulmoary shunt (≥16mmHg) remains a significant risk factor for adverse events after TCPC even though mean pulmonary artery pressure decreased below 10 mmHg before TCPC.