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Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits
  1. Floris E A Udink ten Cate1,2,
  2. Uwe Trieschmann3,
  3. Ingo Germund1,
  4. Tobias Hannes1,
  5. Mathias Emmel1,
  6. Gerardus Bennink4,
  7. Narayanswami Sreeram1
  1. 1 Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany
  2. 2 Department of Paediatric Cardiology, Amalia Children’s Hospital, Radboud University Medical Centre, Nijmegen, The Netherlands
  3. 3 Department of Anaesthesiology and Intensive Care Medicine, University Hospital of Cologne, Cologne, Germany
  4. 4 Department of Cardiac Surgery, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany
  1. Correspondence to Dr Narayanswami Sreeram, Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Kerpenerstrasse 62, 50973 Cologne, Germany; n.sreeram{at}uni-koeln.de

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.

  • Fontan – total cavopulmonary connection – extracardiac conduit –stenosis – protein-losing enteropathy

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