Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits

Heart. 2017 Jul;103(14):1111-1116. doi: 10.1136/heartjnl-2016-310511. Epub 2017 Feb 25.

Abstract

Objectives: An unobstructed extracardiac conduit (ECC) is essential for optimal Fontan haemodynamics. We aimed to evaluate the feasibility and results of percutaneous transcatheter stenting of the ECC in paediatric patients with a significant Fontan pathway obstruction.

Methods: Our institutional database was searched to identify all Fontan patients who had a stent placed in their ECC. Medical records, cardiac catheterisation data and echocardiographic investigations were reviewed. Vessel diameters were normalised to account for differences in body surface area.

Results: Nineteen Fontan patients (age 6.5±3.2 years; male 78.9%) with a significant stenosis of their Dacron ECC graft were identified. Seven patients presented with protein-losing enteropathy (36.8%). An ECC obstruction was suspected on echocardiography in only 6/19 patients (31.6%). The mean minimum diameter of the ECC was 8.3±2.4 mm. A stenosis of >45% was seen in the majority of patients (n=12, 63.1%). Significant correlations between the severity of the ECC obstruction and Fontan pathway vessel diameters were found (all p<0.05). Stenting was successful in all children. The ECC diameter increased significantly after stenting (p<0.0001). An acute clinical benefit of ECC stenting was observed in 18/19 (94.7%) patients. ECC patency was good during a mean follow-up of 1.8±0.9 years.

Conclusions: The feasibility and acute results of percutaneous transcatheter ECC stenting are promising and may provide a good alternative to postpone surgery to a later age. The mechanisms contributing to the development of ECC stenoses are likely multifactorial.

Keywords: Fontan – total cavopulmonary connection – extracardiac conduit –stenosis – protein-losing enteropathy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Angiography
  • Cardiac Catheterization / methods*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Fontan Procedure / adverse effects*
  • Graft Occlusion, Vascular / diagnosis
  • Graft Occlusion, Vascular / surgery*
  • Heart Defects, Congenital / surgery*
  • Humans
  • Infant
  • Male
  • Reoperation
  • Retrospective Studies
  • Stents*