Considerations for Advanced Heart Failure Consultation in Individuals With Fontan Circulation: Recommendations From

Adam M Lubert1, Ari Cedars2, Christopher S Almond3

  • 1Cincinnati Children's Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, OH (A.M.L., A.L., D.L.S.M., L.S.).

Circulation. Heart Failure
|February 14, 2023
PubMed

Insights

Individuals with Fontan circulation face risks of death from heart and non-heart issues. New guidelines from the ACTION Fontan Committee aim to improve timely referrals for advanced heart failure care, addressing a critical gap.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Individuals with Fontan circulation have increased risks of late mortality from cardiac and noncardiac causes.
  • Current referral indications for advanced heart failure care in this population are inconsistent, leading to delayed or missed consultations.
  • This can result in preventable deaths from Fontan circulation-related complications.

Purpose of the Study:

  • To establish clear, evidence-based guidelines for timely referral to advanced heart failure care for individuals with Fontan circulation.
  • To provide primary cardiologists with recommended thresholds for referral based on clinical Fontan circulatory dysfunction.
  • To reduce late mortality by ensuring appropriate and prompt access to advanced therapies.

Main Methods:

  • Development of consensus-based recommendations by the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee.
  • Categorization of clinical Fontan circulatory dysfunction into four key areas: cardiac/systemic ventricular dysfunction, Fontan pathway dysfunction, lymphatic dysfunction, and extracardiac dysfunction.
  • Establishment of specific thresholds within these categories to guide advanced heart failure referral.

Main Results:

  • The ACTION Fontan Committee has proposed specific recommendations for advanced heart failure referral.
  • These recommendations are structured around four categories of Fontan circulatory dysfunction.
  • The guidelines aim to standardize and optimize the timing of consultations for advanced heart failure therapies.

Conclusions:

  • There is a critical need for standardized guidelines for advanced heart failure referral in individuals with Fontan circulation.
  • The ACTION Fontan Committee's recommendations provide a framework to guide cardiologists in identifying patients who require timely consultation.
  • Implementing these guidelines has the potential to improve outcomes and reduce mortality in this high-risk population.

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