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Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes.

Tarek Alsaied1, Runjia Li2, Haley Grant2

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Diastolic dysfunction (DD) after Fontan procedure, indicated by elevated end-diastolic pressure (EDP) >13 mm Hg, significantly increases adverse outcomes. Risk factors include older age, higher BMI, and non-left ventricular morphology.

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Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Patients with single ventricle physiology post-Fontan procedure face high risks of diastolic dysfunction (DD) and elevated end-diastolic pressure (EDP).
  • Early identification and management of DD are crucial for improving long-term outcomes in this population.

Purpose of the Study:

  • To establish the optimal EDP threshold indicative of adverse outcomes following the Fontan procedure.
  • To identify clinical and imaging predictors associated with the development of DD in Fontan patients.

Main Methods:

  • Utilized data from the Fontan Outcome Registry using Cardiac Magnetic Resonance Examinations (FORCE).
  • Included patients who underwent cardiac catheterization and cardiac magnetic resonance (CMR) within a two-year window.
  • Defined a composite outcome including mortality, arrhythmias, plastic bronchitis, protein-losing enteropathy, or transplant listing.

Main Results:

  • Analyzed 861 patients (mean age 16.4 ± 9.3 years); DD was defined at an optimal EDP threshold >13 mm Hg.
  • Diastolic dysfunction (DD) was present in 11.3% of patients and was associated with a >3-fold increased risk of adverse outcomes (HR 3.37).
  • Predictors of DD included older age at catheterization, higher BMI, non-left ventricular morphology, and larger ventricular end-diastolic volume (EDV).

Conclusions:

  • Elevated EDP >13 mm Hg signifies diastolic dysfunction (DD) and is linked to a significantly higher risk of adverse outcomes post-Fontan.
  • Older age, higher BMI, non-left ventricular morphology, and increased EDV are key risk factors for DD.
  • Screening catheterization in patients with risk factors may aid in early DD detection and tailored management strategies.