Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy

Cardiol Young. 2015 Dec;25(8):1489-92. doi: 10.1017/S1047951115002309.


The Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

Keywords: Congenital heart disease; Fontan procedure; physiology; pulmonary vasodilator.

Publication types

  • Review

MeSH terms

  • Bosentan
  • Cardiac Output
  • Endothelin Receptor Antagonists / therapeutic use*
  • Fontan Procedure*
  • Heart Defects, Congenital / surgery*
  • Heart Failure / drug therapy*
  • Heart Failure / physiopathology
  • Humans
  • Phenylpropionates / therapeutic use
  • Phosphodiesterase 5 Inhibitors / therapeutic use*
  • Postoperative Complications / drug therapy*
  • Postoperative Complications / physiopathology
  • Pulmonary Circulation / physiology
  • Pyridazines / therapeutic use
  • Sildenafil Citrate / therapeutic use
  • Sulfonamides / therapeutic use
  • Vascular Resistance
  • Vasodilator Agents / therapeutic use*


  • Endothelin Receptor Antagonists
  • Phenylpropionates
  • Phosphodiesterase 5 Inhibitors
  • Pyridazines
  • Sulfonamides
  • Vasodilator Agents
  • Sildenafil Citrate
  • ambrisentan
  • Bosentan