Fontan Circulation over Time

Djoeke Wolff*, Joost P. van Melle, Beatrijs Bartelds, Floris-Jan S. Ridderbos, Graziella Eshuis, Elisabeth B. H. J. van Stratum, Salvador J. Recinos, Brigitte W. M. Willemse, Hans Hillege, Tineke P. Willems, Tjark Ebels, Rolf M. F. Berger

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

7 Citations (Scopus)

Abstract

The unique, unphysiological Fontan circulation is associated with an impaired functional status of the patients that is suggested to deteriorate over time. Unfortunately, previous studies did not integrate both pulmonary and cardiac determinants of functional status. In addition, a comparison with the natural decrease in exercise capacity in healthy subjects (in both children and adults) is lacking. This single-center study aims to investigate the functional status in a cohort of Fontan patients in relation to time since Fontan completion and to identify its determinants, including cardiac characteristics and pulmonary characteristics. Eighty-five consecutive Fontan patients z10 years who performed adequate cardiopulmonary exercise testing (respiratory exchange ratio >1.01) were included. Mean time since Fontan completion was 15 9 years (range 2 to 37 years). New York Heart Association functional class was I in 36 patients (42%), II in 41 patients (48%), and III in 8 patients (9%). Peak oxygen uptake during exercise (VO2 index) was 25.7 +/- 7.9 mllmin/m(2) (58 14% of predicted). New York Heart Association functional class and peak VO2 index both correlated with time since the Fontan operation; however, peak VO2 as percentage of predicted (VO2(pred)) did not. In multivariate analyses, peak VO2(pred) was independently associated with maximum heart rate, oxygen pulse at peak exercise, and forced expiratory volume in 1 second (R-2 = 0.579) but not with cardiac output in rest. In conclusion, the present data suggest that functional status in Fontan patients is impaired already shortly after Fontan completion, whereas its subsequent deterioration seems to follow the natural decline of aging. Furthermore, functional status in Fontan patients correlates with pulmonary function and cardiac functional parameters during exercise but not with conventional cardiac measurements at rest.

Original languageEnglish
Pages (from-to)461-466
Number of pages6
JournalAmerican Journal of Cardiology
Volume120
Issue number3
DOIs
Publication statusPublished - 1-Aug-2017

Keywords

  • CONGENITAL HEART-DISEASE
  • LUNG-FUNCTION
  • PULMONARY-FUNCTION
  • TRICUSPID-ATRESIA
  • REFERENCE VALUES
  • EXERCISE
  • OPERATION
  • MULTICENTER
  • MORTALITY
  • CAPACITY

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