title: Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy.
abstract: 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.
authors.affiliation: 1The Cardiac Center,The Children's Hospital of PhiladelphiaPhiladelphia,Pennsylvania,United States of America.
authors.forename: Brian S
authors.initials: BS
authors.lastname: Snarr
authors.affiliation: 1The Cardiac Center,The Children's Hospital of PhiladelphiaPhiladelphia,Pennsylvania,United States of America.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Paridon
authors.affiliation: 1The Cardiac Center,The Children's Hospital of PhiladelphiaPhiladelphia,Pennsylvania,United States of America.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: 1The Cardiac Center,The Children's Hospital of PhiladelphiaPhiladelphia,Pennsylvania,United States of America.
authors.forename: David J
authors.initials: DJ
authors.lastname: Goldberg
mesh_terms: D000077300:Bosentan; D002302:Cardiac Output; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000601:surgery*; D006333:Heart Failure / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D010666:Phenylpropionates / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D011183:Postoperative Complications / Q000188:drug therapy* / Q000503:physiopathology; D011652:Pulmonary Circulation / Q000502:physiology; D011724:Pyridazines / Q000627:therapeutic use; D000068677:Sildenafil Citrate / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D065128:Endothelin Receptor Antagonists; D010666:Phenylpropionates; D058986:Phosphodiesterase 5 Inhibitors; D011724:Pyridazines; D013449:Sulfonamides; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; C467894:ambrisentan; D000077300:Bosentan
keywords: Congenital heart disease; Fontan procedure; physiology; pulmonary vasodilator