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

Author:

Lubert Adam M.1ORCID,Cedars Ari2ORCID,Almond Christopher S.3,Amdani Shahnawaz4ORCID,Conway Jennifer5ORCID,Friedland-Little Joshua M.6ORCID,Gajarski Robert J.7ORCID,Kindel Steven J.8ORCID,Lorts Angela1,Morales David L.S.1ORCID,O’Connor Matthew J.9,Peng David M.10ORCID,Rosenthal David N.3ORCID,Smyth Lauren1,Sutcliffe David L.11ORCID,Schumacher Kurt R.10ORCID

Affiliation:

1. Cincinnati 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.).

2. Johns Hopkins University, Baltimore, MD (A.C.).

3. Stanford University School of Medicine (Pediatrics) and Stanford Children’s Health, Palo Alto, CA (C.S.A., D.N.R.).

4. Cleveland Clinic Children’s, OH (S.A.).

5. Stollery Children’s Hospital, Edmonton, Alberta, Canada (J.C.).

6. Seattle Children’s Hospital, WA (J.M.F.-L.).

7. Nationwide Children’s Hospital, Columbus, OH (R.J.G.).

8. Children’s Hospital of Wisconsin and Herma Heart Institute, Medical College of Wisconsin, Milwaukee (S.J.K.).

9. Children’s Hospital of Philadelphia, PA (M.J.O.).

10. Congenital Heart Center, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor (D.M.P., K.R.S.).

11. Children’s Mercy Hospital, Kansas City, MO (D.L.S.).

Abstract

Individuals with Fontan circulation are at risk of late mortality from both cardiac and noncardiac causes. Despite the known risk of mortality, referral indications for advanced heart failure care vary between centers, and many individuals die from Fontan circulation-related complications either after late consideration for advanced heart failure therapies or having never seen a heart failure specialist. There is a critical need for guidelines to direct appropriately timed referral for advanced heart failure consultation. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Fontan Committee has developed recommended thresholds for advanced heart failure referral to guide primary cardiologists. These recommendations are divided into 4 categories of clinical Fontan circulatory dysfunction including (1) cardiac/systemic ventricular dysfunction, (2) Fontan pathway dysfunction, (3) lymphatic dysfunction, and (4) extracardiac dysfunction.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Cardiology and Cardiovascular Medicine

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