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

Cardiol Young. 2011 Dec:21 Suppl 2:77-9. doi: 10.1017/S1047951111001636.

Abstract

Children with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart.

Publication types

  • Review

MeSH terms

  • Child
  • Fontan Procedure*
  • Heart Defects, Congenital / mortality*
  • Heart Defects, Congenital / surgery
  • Heart Ventricles / abnormalities*
  • Humans
  • Postoperative Period
  • Program Development / methods*
  • Risk Factors
  • Survival Analysis
  • Survival Rate