Long Term Consequences of the Fontan Procedure and How to Manage Them

Prog Cardiovasc Dis. 2018 Sep-Oct;61(3-4):365-376. doi: 10.1016/j.pcad.2018.09.005. Epub 2018 Sep 17.

Abstract

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.

Keywords: Adult congenital heart disease; Fontan.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Fontan Procedure* / adverse effects
  • Fontan Procedure* / methods
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Heart Ventricles / abnormalities
  • Hemodynamics
  • Humans
  • Long Term Adverse Effects* / etiology
  • Long Term Adverse Effects* / physiopathology
  • Long Term Adverse Effects* / therapy
  • Patient Care Management* / methods
  • Patient Care Management* / organization & administration