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Developing an adolescent and adult Fontan Management Programme

Part of: Functionally Univentricular Heart

Published online by Cambridge University Press:  10 May 2021

Adam M. Lubert Open the ORCID record for Adam M. Lubert [Opens in a new window] ,
Tarek Alsaied Open the ORCID record for Tarek Alsaied [Opens in a new window] ,
Andrew T. Trout ,
Jonathan R. Dillman ,
Joseph J. Palermo ,
Felicia Eichelbrenner ,
Kelly Kleier ,
Angela Lorts ,
Nadeem Anwar  and
Marc G. Schecter
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Adam M. Lubert*
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Cincinnati Adult Congenital Heart Disease Program, Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Tarek Alsaied
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Andrew T. Trout
Affiliation:
Cincinnati Children’s Hospital Medical Center, Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Jonathan R. Dillman
Affiliation:
Cincinnati Children’s Hospital Medical Center, Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Joseph J. Palermo
Affiliation:
Cincinnati Children’s Hospital Medical Center, Division of Gastroenterology, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Felicia Eichelbrenner
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Kelly Kleier
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Angela Lorts
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Nadeem Anwar
Affiliation:
Digestive Disease Division, Department of Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Marc G. Schecter
Affiliation:
Cincinnati Children’s Hospital Medical Center, Division of Pulmonology, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Gregory M. Tiao
Affiliation:
Division of Transplant Surgery, Department of Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
Clifford Chin
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
David S. Feldman
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Division of Cardiovascular Medicine, Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA
Meredith Jenkins
Affiliation:
Division of Pharmacy, Heart Institute, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
David LS Morales
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Surgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Alexander R. Opotowsky
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Cincinnati Adult Congenital Heart Disease Program, Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
John C. Bucuvalas
Affiliation:
Recanati-Miller Transplant Institute and the Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, USA
Gruschen R. Veldtman
Affiliation:
Adult Congenital Heart Disease, Department of Pediatrics, Cincinnati Children’s Hospital and Heart Centre and King Faisal Specialist Hospital and Research Centre, Riyadh45229, Saudi Arabia
Stuart L. Goldstein
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Division of Nephrology and Hypertension, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
*
Author for correspondence: Adam M. Lubert, MD, Department of Pediatrics, Cincinnati Children’s Hospital, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH45229-3026, USA. Tel: 513-803-2243; Fax: 513-803-0079. E-mail: adam.lubert@cchmc.org
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Abstract

Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

Keywords

Fontanmultidisciplinary clinicadult congenital heart disease
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 2 , February 2022 , pp. 230 - 235
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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References

Egbe, AC, Poterucha, JT, Warnes, CA et al. Hepatocellular Carcinoma After Fontan Operation. Circulation 2018; 138: 746–748.CrossRefGoogle ScholarPubMed
Di Maria, MV, Barrett, C, Rafferty, C et al. Initiating a Fontan multidisciplinary clinic: decreasing care variability, improving surveillance, and subsequent treatment of Fontan survivors. Congenit Heart Dis 2019; 14: 590–599.CrossRefGoogle ScholarPubMed
Downing, TE, Allen, KY, Glatz, AC et al. Long-term survival after the Fontan operation: twenty years of experience at a single center. J Thorac Cardiovasc Surg 2017; 154: 243–253.e242.CrossRefGoogle ScholarPubMed
Gurvitz, M, Valente, AM, Broberg, C et al. Prevalence and predictors of gaps in care among adult congenital heart disease patients. J Am Coll Cardiol 2013; 61: 2180–2184.CrossRefGoogle ScholarPubMed
Dillman, JR, Trout, AT, Alsaied, T, Gupta, A, Lubert, AM Imaging of Fontan-associated liver disease. Pediatr Radiol 2020; 50: 1528–1541.CrossRefGoogle ScholarPubMed
Schumacher, KR, Kindel, SJ ACTION Committee: Considerations for Advanced Heart Failure Consultation in Fontan Patients.Google Scholar
Rychik, J, Atz, AM, Celermajer, DS et al. Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation 2019: Cir0000000000000696.CrossRefGoogle Scholar
Khairy, P, Fernandes, SM, Mayer, JE Jr. et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008; 117: 85–92.CrossRefGoogle ScholarPubMed
Alsaied, T, Allen, KY, Anderson, JB et al. The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States. Cardiol Young 2020: 1–6.Google Scholar