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New concepts: development of a survivorship programme for patients with a functionally univentricular heart

David J Goldberg1, Kathryn Dodds, Jack Rychik

  • 1The Cardiac Center, The Children's Hospital of Philadelphia, 34th Street & Civic Center Boulevard, Philadelphia, PA 19146, USA. goldbergda@email.chop.edu

Cardiology in the Young
|December 14, 2011
PubMed

Insights

Long-term survivors of the Fontan operation for functionally univentricular hearts face significant health challenges. Multidisciplinary care is essential to manage complications and improve quality of life for these Fontan patients.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Fontan Physiology

Background:

  • Increasing survival rates for children with functionally univentricular hearts into adulthood.
  • The Fontan operation, while life-saving, presents long-term health challenges.
  • Need for improved quality and duration of life post-Fontan operation.

Purpose of the Study:

  • To highlight the complex, long-term complications following the Fontan operation.
  • To emphasize the necessity of specialized, multidisciplinary care for Fontan survivors.
  • To advocate for integrated clinical services to address Fontan-associated morbidities.

Main Methods:

  • Review of known complications associated with Fontan physiology.
  • Discussion of the limitations in current pediatric cardiology training regarding Fontan sequelae.
  • Emphasis on the importance of collaborative care models involving various subspecialists.

Main Results:

  • Fontan survivors face risks including liver disease, low bone density, protein-losing enteropathy, and plastic bronchitis.
  • Pediatric cardiologists require enhanced understanding of Fontan pathophysiology and treatment options.
  • Collaboration with gastroenterologists, endocrinologists, and pulmonologists is crucial.

Conclusions:

  • Optimal care for Fontan survivors necessitates a comprehensive, multidisciplinary approach.
  • Specialized clinics offering integrated subspecialist consultations can significantly benefit patients.
  • Addressing the complex needs of Fontan patients improves long-term outcomes and quality of life.

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