Perioperative Treprostinil in Pediatric Patients Undergoing the Fontan Operation
Status:
Completed
Trial end date:
2020-12-01
Target enrollment:
Participant gender:
Summary
The purpose of this study is to test the efficacy of treprostinil in the perioperative
non-fenestrated, extracardiac Fontan patients in order to reduce duration of chest tube
drainage (in days).
The Fontan operation is performed for patients with single ventricle physiology as the final
palliation to create a series circulation, with passive systemic venous return to the
pulmonary arteries and the single ventricle solely providing systemic output.
Patients undergoing extracardiac Fontan tend to have elevation of Fontan pressures
immediately following the operation with inflammation from surgery requiring additional fluid
administration to maintain blood pressure. Increased Fontan pressures and fluid overload lead
to prolonged chest tube drainage.
The hypothesis is that treprostinil, a prostacyclin drug that dilates the pulmonary arteries,
will improve immediate postoperative Fontan pressures. Treprostinil is not FDA approved for
this use. Anecdotally and in a small case series, prostacyclin therapy has been shown to
assist in transitioning patients off nitric oxide. The investigators believe that this
improvement in hemodynamics will decrease duration of chest tube drainage resulting in a
shorter length of hospital stay.