Each 1-SD increase in pre-Fontan mean pulmonary arterial pressure is associated with a 2.2-fold higher hazard of death or transplantation after discharge (HR 2.20).
Does elevated pre-Fontan mean pulmonary arterial pressure predict long-term risk of death or transplantation in pediatric patients undergoing Fontan palliation?
Elevated pre-Fontan mean pulmonary arterial pressure, systemic right ventricle, and delayed Fontan completion (>4 years of age) are strong independent predictors of long-term mortality or transplantation up to 25 years after Fontan palliation.
Absolute Event Rate: 0% vs 0%
BACKGROUND: Long-term outcomes after Fontan vary widely. Although pre-Fontan hemodynamics predict early failure, their association with long-term outcomes remains unclear. We hypothesized that pre-Fontan hemodynamics predict long-term risk of death or transplantation. METHODS: We analyzed data from the Pediatric Cardiac Care Consortium, a US-based multicenter registry including patients undergoing first-time Fontan with pre-Fontan catheterization and long-term follow-up. Patients undergoing a Fontan procedure before 18 years of age at any time between 1982 and 2011 were included in the study. Outcomes of interest were in-hospital Fontan failure (death or takedown) and postdischarge death or transplantation, identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022. Associations between pre-Fontan hemodynamics and long-term risk for death or transplantation were assessed with Kaplan-Meier survival curves and extended Cox regression. RESULTS: Among 1175 patients (736 62.6% male, 626 53.3% with systemic left ventricle), 1111 were discharged with Fontan physiology. Over a median postdischarge follow-up of 20.6 years (interquartile range, 18.2–24.4 years), 85 deaths and 49 transplantations occurred. Pre-Fontan mean pulmonary arterial pressure was the strongest hemodynamic predictor of postdischarge outcomes with a continuous association and no clear inflection point; 25-year transplantation-free survival declined from 83.7% (95% CI, 77.6–88.3) in the low mean pulmonary arterial pressure tertile to 73.7% (95% CI, 65.5–80.3) in the highest tertile (log-rank P =0.02). Each 1-SD increase in mean pulmonary arterial pressure was associated with 1.33-fold higher odds of in-hospital failure (adjusted odds ratio, 1.33 95% CI, 1.00–1.77; P =0.05) and a 2.2-fold higher hazard of death or transplantation (adjusted hazard ratio, 2.20 95% CI, 1.62–3.00; P 4 years of age versus 2 to 4 years of age; adjusted hazard ratio, 1.80 95% CI, 1.25–2.60; P =0.02). CONCLUSIONS: Elevated pre-Fontan mean pulmonary arterial pressure is a strong predictor of in-hospital and long-term post-Fontan risk of death or transplantation. Systemic right ventricle and delayed Fontan completion (>4 years of age) further increased risk. These findings support early Fontan consideration and ongoing hemodynamic surveillance to optimize long-term outcomes.
Suthar et al. (Thu,) reported a other. Each 1-SD increase in pre-Fontan mean pulmonary arterial pressure is associated with a 2.2-fold higher hazard of death or transplantation after discharge (HR 2.20).