The Fontan Outcomes Network: Findings After 2 Years and 1121 Participants
Abstract
BACKGROUND: Survival after Fontan palliation for single ventricle heart disease has improved substantially, yet the long-term trajectory remains poorly defined. The Fontan Outcomes Network, a learning health network of 38 congenital heart centers in the United States and Canada, was established to address this gap. We report baseline characteristics and early findings from the first 1121 participants enrolled in this prospective clinical registry. METHODS: We performed a cross-sectional analysis of individuals who had undergone Fontan palliation enrolled in the Fontan Outcomes Network from August 2022 through August 2024. Demographic, clinical, imaging, procedural, and medication data were analyzed descriptively, overall and by age group (<12, 12 to <18, and ≥18 years). RESULTS: A total of 1121 participants were enrolled (mean age, 16.3±10.2 years; 471 [42%] female). Hypoplastic left heart syndrome (n=431 [38.5%]) and right ventricular–dominant anatomy (n=615 [54.9%]) were the most common primary cardiac diagnoses, especially in younger age groups. Extracardiac conduit was the most frequent Fontan type (n=749 [66.8%]). Typical Fontan-related adverse events were noted in 59% of cases (n=662), including chylous pleural effusions (n=108 [9.6%]), Fontan thrombosis (n=79 [7.1%]), and nonperioperative stroke (n=73 [6.5%]). A history of arrhythmia was present in 41.3% of patients (n=463) overall, increasing with age. Acquired comorbidities were present in 57.5% of patients (n=645), including asthma (n=132 [12%]) and sleep apnea (n=103 [9%]), with burden increasing by age. Antithrombotic medication use was nearly universal. Use of other medications varied widely by age, and included β-blockers, angiotensin-converting enzyme inhibitors, and pulmonary vasodilators. A clinical diagnosis of an anxiety disorder was present in 34.1% of patients (n=382), including 48.1% of adults (n=194 of 403), and antidepressants were prescribed in 11.8% of patients (n=132), including 23.6% of adults (n=95 of 403), highlighting the mental health needs of this population. CONCLUSIONS: The Fontan Outcomes Network includes one of the largest prospective, multi-institutional data sets of patients with Fontan circulation. These initial findings demonstrate the potential for a collaborative learning health network to advance research and quality improvement for this rare disease population.
Article Details
Authors (87)
Thomas M. Glenn
Pediatrics and Cardiology, Baylor College of Medicine and Texas Children’s Hospital, Houston (T.M.G., S.J.D.).
Nadine A. Kasparian
Shahnawaz Amdani
David Renaud
Department of Child and Adolescent Health, Division of Pediatrics III–Cardiology, Pulmonology, Allergology and Cystic Fibrosis, Medical University of Innsbruck, Austria (D.R.).
Michael V. DiMaria
Pediatrics and Cardiology, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor (M.V.D., K.R.S.; M.V.D. now with HCA HealthONE Rocky Mountain Children’s at Presbyterian St. Luke’s, Denver, CO).
Jennifer Teh
Heart Institute (J. Teh, N.A.P.), Children’s Hospital Los Angeles, CA.
David M. Leone
Heart Institute (N.A.K., D.M.L., R.V., J. Theobald, J.B.A., A.R.O.)
Arjun K. Mahendran
Pediatrics, Texas Center for Pediatric and Congenital Heart Disease, Dell Medical School at The University of Texas at Austin/Dell Children’s Medical Center (A.K.M.).
Nancy A. Pike
Heart Institute (J. Teh, N.A.P.), Children’s Hospital Los Angeles, CA.
Stephen J. Dolgner
Pediatrics and Cardiology, Baylor College of Medicine and Texas Children’s Hospital, Houston (T.M.G., S.J.D.).
Andrew L. Cheng
Pediatrics and Cardiology (A.L.C.), Children’s Hospital Los Angeles, CA.
Rahul H. Rathod
Sumeet S. Vaikunth
Internal Medicine and Pediatrics, Hospital of the University of Pennsylvania, Philadelphia (S.S.V.).
Surendranath R. Veeram Reddy
Pediatrics and Cardiology, Children’s Health, UT Southwestern, Dallas, TX (S.R.V.R.).
Rohith Vanam
Heart Institute (N.A.K., D.M.L., R.V., J. Theobald, J.B.A., A.R.O.)
Jeff Theobald
Heart Institute (N.A.K., D.M.L., R.V., J. Theobald, J.B.A., A.R.O.)
Kurt R. Schumacher
Pediatrics and Cardiology, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor (M.V.D., K.R.S.; M.V.D. now with HCA HealthONE Rocky Mountain Children’s at Presbyterian St. Luke’s, Denver, CO).
Sarah A. Hummel
Jennifer Conway
David J. Goldberg
Pediatrics (D.J.G.), Children’s Hospital of Philadelphia, PA.
Dala Zakaria
Pediatrics and Cardiology, Arkansas Children’s Hospital, Little Rock (D.Z.).
Sarah B. Clauss
Pediatrics and Cardiology, Children’s National Hospital, Washington, DC (S.B.C.).
Divya Suthar
Pediatrics and Cardiology, Children’s Healthcare of Atlanta, GA (D.S.).
Roni M. Jacobsen
Pediatrics and Cardiology, Children’s Hospital Colorado, Aurora (R.M.J.).
Evonne M. Morell
Pediatrics and Cardiology, Children’s Hospital of Pittsburgh of UPMC, PA (E.M.M., T.A.; T.A. now with Pediatrics and Cardiology, Children’s Health, UT Southwestern, Dallas, TX).
John C. Dykes
Pediatrics and Cardiology, Lucile Packard Children’s Hospital, Stanford University, Palo Alto, CA (J.C.D.).
Angela J. Weingarten
Medicine and Pediatrics, Divisions of Cardiovascular Medicine and Pediatric Cardiology, Monroe Carrell Jr Children’s Hospital, Vanderbilt University Medical Center, Nashville, TN (A.J.W.).
Deepti P. Bhat
Pediatrics and Cardiology, Phoenix Children’s Hospital, AZ (D.P.B.).
Benjamin E. Reinking
Pediatrics and Cardiology, University of Iowa Stead Children’s Hospital, Iowa City (B.E.R.).
Tarek Alsaied
Carole Lannon
James M. Anderson Center for Health Systems Excellence (C.L.)
Kiona Y. Allen
Pediatrics and Cardiology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern Feinberg School of Medicine, Chicago, IL (K.Y.A.).
Jeff B. Anderson
Heart Institute (N.A.K., D.M.L., R.V., J. Theobald, J.B.A., A.R.O.)
Lacie B. Patterson
Sisters by Heart, Valley Center, CA (L.B.P.).
David W. Brown
Pediatrics and Cardiology, Boston Children’s Hospital, MA (R.H.R., D.W.B.).
Richard James
Medical Education, Library Services, Nemours Children’s Health, Wilmington, DE (R.J.).
Jack Rychik
Cardiology (D.J.G., J.R.), Children’s Hospital of Philadelphia, PA.
Alexander R. Opotowsky
Heart Institute (N.A.K., D.M.L., R.V., J. Theobald, J.B.A., A.R.O.)
Jeremy T. Affolter
Diane Aguero
Alaa Aljiffry
Saleem Almasarweh
Julie Bonn
Jamie N. Colombo
Paul Critser
Meghan A. Didier
Kathryn M. Dodds
Francine Erenberg
David M. Finkelstein
Therese M. Giglia
Salil Ginde
Molly Gord
Joseph N. Graziano
Jessica Haley
Stephanie S. Handler
Jesse Hansen
Anyir Hsieh
Alicia K. Hutson
Natalie M. Jayaram
Omar M. Khalid
Matthew S. Lemler
Andrew S. Mackie
Amanda McCormick
Kimberly L. Moore
Lakshmi Nagaraju
Melanie Parent
Trudy Pierick
Michelle S. Ploutz
Pierangelo Renella
Kate Robb
Nancy Rudd
Ilana Schwartz
Kavisha M. Shah
Rachel J. Shustak
Jose Silva Sepulveda
Megan Soohoo
Eileen Stewart
Michelle M. Steltzer
Sherzana Sunderji
Karen M. Texter
Rocky Tsang
Casey Vogel
Jodie K. Votava-Smith
Justin Weigand
Alicia N. Wilmoth
Fred Wu
Adel K. Younoszai