Title:Current Treatment Options for the Failing Fontan Circulation
Volume: 18
Issue: 4
Author(s): Bart W. Driesen, Michiel Voskuil and Heynric B. Grotenhuis*
Affiliation:
- Department of Pediatric Cardiology, Wilhelmina Children’s Hospital, University Medical Center, Utrecht, Utrecht,
The Netherlands
Keywords:
Pediatrics, heart defects, congenital, fontan, complications, treatment.
Abstract: The Fontan operation was introduced in 1968. For congenital malformations, where
biventricular repair is unsuitable, the Fontan procedure has provided a long-term palliation strategy
with improved outcomes compared to the initially developed procedures. Despite these improvements,
several complications merely due to a failing Fontan circulation, including myocardial dysfunction,
arrhythmias, increased pulmonary vascular resistance, protein-losing enteropathy, hepatic
dysfunction, plastic bronchitis, and thrombo-embolism, may occur, thereby limiting the life-expectancy
in this patient cohort. This review provides an overview of the most common complications
of Fontan circulation and the currently available treatment options.