Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may

Kirsten C Odegard1, Francis X McGowan, David Zurakowski

  • 1Department of Anesthesia, Children's Hospital, Boston, MA 02115, USA. kirsten.odegard@tch.harvard.edu

Insights

Children after the Fontan operation show lower levels of key coagulation factors. Elevated factor VIII in some patients warrants further investigation for thrombosis risk and potential anticoagulation therapy.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Coagulation Science

Background:

  • The Fontan operation is a palliative procedure for complex congenital heart disease.
  • Coagulation abnormalities are a known complication in Fontan patients.
  • Hemodynamic changes post-Fontan may influence coagulation factor levels.

Purpose of the Study:

  • To prospectively evaluate coagulation factor abnormalities in children post-Fontan operation.
  • To assess the relationship between hemodynamic variables and coagulation status.
  • To identify potential predictors of coagulation abnormalities in this population.

Main Methods:

  • Assayed coagulation factors in 20 children post-Fontan and 24 age-matched controls.
  • Measured concentrations of multiple coagulation factors, inhibitors, and liver enzymes.
  • Evaluated demographic, hemodynamic, and time-related variables as predictors.

Main Results:

  • Fontan patients exhibited significantly lower levels of protein C, factors II, V, VII, X, plasminogen, and ATIII compared to controls.
  • Factor VIII was elevated in 35% of Fontan patients, with two experiencing thromboembolic events.
  • Higher superior vena cava pressure predicted elevated factor VIII, but no other hemodynamic factors predicted coagulation abnormalities.

Conclusions:

  • Procoagulant and anticoagulant factor levels are generally reduced in Fontan survivors, irrespective of Fontan-specific hemodynamics.
  • Elevated factor VIII levels in Fontan patients require further study as a potential cause of thrombosis.
  • Identifying patients with elevated factor VIII may guide decisions regarding anticoagulation therapy.
Abstract

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