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Objectives: Long-term data on the impact of pulmonary artery (PA) augmentation in patients who underwent the Fontan procedure are lacking. The aim of this study was to examine whether surgical or transcatheter PA augmentation at or prior to the Fontan procedure adversely affects the outcomes.
Methods: Data of 1436 patients from the Australia-New Zealand Fontan Registry (1975-2015) were analysed. Primary end point was death or Fontan failure. Cox regression with propensity score matching was used to determine risk or benefit conferred by PA augmentation.
Results: Forty-eight (3.3%) patients underwent PA augmentation following cavopulmonary shunt (surgical n = 14, balloon dilatation n = 16 and stent n = 18) and 105 (7.3%) patients underwent PA augmentation at the time of the Fontan procedure (surgical n = 104, stent n = 1). Median follow-up was 6.4 years with 10 deaths (6.5%) in the augmentation group and 10.5 years with 95 deaths (7.4%) in the non-augmentation group. The unadjusted Kaplan-Meier, log-rank test and Cox regression analysis demonstrated no significant difference in both end points between the 2 groups [death: hazard ratio (HR) 1.35, 95% confidence interval (CI) 0.70-2.60; P = 0.37 death or failure: HR 1.39, 95% CI 0.83-2.34; P = 0.21]. The propensity score matching yielded 131 matched pairs, with adequate balance for all covariates (the median residual bias = 0.05). The subsequent Cox regression demonstrated no significant difference in the risks of death (HR 1.30, 95% CI 0.49-3.41; P = 0.60) and death or Fontan failure (HR 0.92, 95% CI 0.46-1.85; P = 0.82).
Conclusions: PA augmentation prior to or at the Fontan procedure does not confer worse long-term outcomes.
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http://dx.doi.org/10.1093/ejcts/ezy376 | DOI Listing |
Aims: Many patients develop Fontan-associated liver disease (FALD) after undergoing the Fontan procedure-a surgical treatment for congenital heart disease such as single ventricle-owing to changes in venous pressure and cardiac output. Liver biopsy is the gold standard for diagnosing FALD, but has limitations. Magnetic resonance elastography (MRE) is a popular non-invasive method for evaluating liver stiffness and fibrosis in FALD; however, no unified view exists.
View Article and Find Full Text PDFInterv Radiol (Higashimatsuyama)
July 2025
Department of Radiology, Wakayama Medical University, Japan.
Purpose: We retrospectively analyzed the effect of coil type on the number of coils used and the procedure time in pre-Fontan coil embolization of collateral arteries.
Material And Methods: Twelve patients with congenital heart disease underwent coil embolization before Fontan surgery between 2010 and 2021. They were divided into 2 groups.
Pediatr Transplant
November 2025
D'Or Institute for Research and Education (IDOR), Rio de Janeiro, RJ, Brazil.
Background: Fontan-associated liver disease can progress to advanced fibrosis, raising the potential need for combined heart-liver transplantation (CHLT) in selected patients. However, the benefits of CHLT over isolated orthotopic heart transplantation (HT), particularly in terms of mortality, remain uncertain. In this systematic review, we compared mortality outcomes following CHLT versus HT in patients with Fontan circulation, with the aim of supporting clinical decision-making.
View Article and Find Full Text PDFCardiol Young
August 2025
Division of Cardiology and multi-disciplinary Fontan clinic, Phoenix Children's' Hospital, Phoenix, AZ, USA.
Background: Implantable haemodynamic monitors allow remote monitoring of Fontan circulation. We report unique opportunities and challenges related to device use in rural, high-altitude regions.
Objectives: Assess the performance of implantable haemodynamic monitor in Fontan circulation and identify potential sources of measurement discrepancy defined as non-physiological, negative, or significantly lower reading than baseline.
Eur Heart J Open
July 2025
Pediatric Heart Center, Department of Pediatric Cardiology, Intensive Care Medicine and Congenital Heart Disease, Justus-Liebig University Giessen, Feulgenstr. 10 - 12, Giessen 35392, Germany.