The Enigma of the Fontan circulation

Wolff, D., 2016, [Groningen]: Rijksuniversiteit Groningen. 247 p.

Research output: ThesisThesis fully internal (DIV)Academic

Copy link to clipboard


  • Title and contents

    Final publisher's version, 207 KB, PDF document

  • Chapter 1

    Final publisher's version, 230 KB, PDF document

  • Chapter 2

    Final publisher's version, 519 KB, PDF document

  • Chapter 3

    Final publisher's version, 456 KB, PDF document

  • Chapter 4

    Final publisher's version, 545 KB, PDF document

  • Chapter 5

    Final publisher's version, 517 KB, PDF document

  • Chapter 6

    Final publisher's version, 622 KB, PDF document

  • Chapter 7

    Final publisher's version, 852 KB, PDF document

  • Chapter 8

    Final publisher's version, 727 KB, PDF document

  • Chapter 9

    Final publisher's version, 963 KB, PDF document

  • Chapter 10

    Final publisher's version, 1 MB, PDF document

  • Chapter 11

    Final publisher's version, 1 MB, PDF document

  • Appendices

    Final publisher's version, 317 KB, PDF document

  • Complete thesis

    Final publisher's version, 3 MB, PDF document

  • Propositions

    Final publisher's version, 62 KB, PDF document

  • Djoeke Wolff
Children who are born with a complex congenital heart disease, with only one well-developed ventricle and one underdeveloped or absent ventricle, usually require a surgical intervention at a young age. The Fontan procedure is designed to treat patients with this so-called functionally univentricular heart (one-chamber-heart) and who are not suitable for a biventricular (two-chamber) repair. The procedure results in a unique, unphysiologic circulation, in which only one ventricle sustains both the pulmonary and the circulatory circulation. Attrition of the Fontan circulation, most likely related to the unphysiologic characteristics, is an emerging problem currently faced by a growing cohort of patients. In this thesis, we demonstrated that the outcome after the Fontan operation has improved over the past decades. However, we also identified that the Fontan circulation is still associated with a multiple sequelae, including limited exercise tolerance, restricted cardiac and pulmonary function, and pulmonary vascular remodeling. Furthermore, with the help of a newly applied magnetic resonance technique, we recognized that the development of liver disease is far more common in Fontan patients than previously thought. Finally, we demonstrated that the surgical treatment for eventual failure of the circulation is associated with considerable mortality, and does not favor one surgical option above another. In conclusion, survival after the Fontan procedure has improved over the past decades, and the exercise tolerance, a biomarker called NT-proBNP and the development of liver disease may all be potential markers of Fontan attrition in the future.
Original languageEnglish
QualificationDoctor of Philosophy
Awarding Institution
  • Berger, Rolf, Supervisor
  • Ebels, Tjark, Supervisor
  • Melle, van, Joost, Co-supervisor
  • Helbing, W.A., Assessment committee, External person
  • Budts, W., Assessment committee, External person
  • Haas, F., Assessment committee, External person
Award date29-Jun-2016
Place of Publication[Groningen]
Print ISBNs978-90-367-8938-7
Electronic ISBNs978-90-367-8937-0
Publication statusPublished - 2016

Download statistics

No data available

ID: 33001862