Journal article
Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium
Circulation (New York, N.Y.)
01/22/2026
DOI: 10.1161/CIRCULATIONAHA.125.076880
PMID: 41568447
Abstract
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.
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.
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
=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];
=0.05) and a 2.2-fold higher hazard of death or transplantation (adjusted hazard ratio, 2.20 [95% CI, 1.62-3.00];
<0.01) estimated at discharge. This hazard declined 3% per year after discharge (adjusted hazard ratio per year, 0.97 [95% CI, 0.95-0.99];
<0.01) and resolved by 17 years in patients with systemic right ventricle and by 23 years in those with systemic left ventricle. Additional independent risk factors included systemic right ventricle versus systemic left ventricle (adjusted hazard ratio, 2.39 [95% CI, 1.65-3.46];
<0.01) and delayed Fontan completion (>4 years of age versus 2 to 4 years of age; adjusted hazard ratio, 1.80 [95% CI, 1.25-2.60];
=0.02).
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.
Details
- Title: Subtitle
- Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium
- Creators
- Divya Suthar - Children's Healthcare of AtlantaYanxu Yang - Emory UniversityAsaad G Beshish - Children's Healthcare of AtlantaJessica Knight - University of GeorgiaXiao Song - University of GeorgiaAmanda Thomas - University of MinnesotaHua Hao - Emory UniversityFawwaz R Shaw - Children's Healthcare of AtlantaKathy Jenkins - Boston Children's HospitalJeffrey P Jacobs - Florida CollegeMatthew E Oster - Children's Healthcare of AtlantaAlvaro Alonso - University of Missouri–Kansas CityYijian Huang - Emory UniversityGeetha Raghuveer - University of Missouri–Kansas CityGurumurthy HiremathBradley Marino - Cleveland ClinicLydia Wright - Nationwide Children's HospitalShriprasad R Deshpande - Children's NationalAnitha S John - Children's NationalMansi M Gaitonde - Children's Medical Center University of Texas Southwestern, Dallas (M.M.G.)Bahaaldin Alsoufi - Norton HospitalDavid M Overman - Children's MinnesotaCharles Canter - St. Louis Children's HospitalJames St Louis - Augusta UniversityRajiv Devanagondi - University of IowaKimberly E McHugh - Medical University of South CarolinaLazaros Kochilas - Emory University
- Resource Type
- Journal article
- Publication Details
- Circulation (New York, N.Y.)
- DOI
- 10.1161/CIRCULATIONAHA.125.076880
- PMID
- 41568447
- ISSN
- 1524-4539
- eISSN
- 1524-4539
- Grant note
- R21 HL145486 / NHLBI NIH HHS R01 HL122392 / NHLBI NIH HHS
- Language
- English
- Electronic publication date
- 01/22/2026
- Academic Unit
- Cardiology; Stead Family Department of Pediatrics
- Record Identifier
- 9985130538902771
Metrics
1 Record Views