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.
In order to understand the effect of treprostinil on Fontan patients, this study has two
1. reactivity testing with inhaled treprostinil in the cardiac catheterization lab for
pre-Fontan patients undergoing routine catheterization in anticipation of the Fontan
2. perioperative use of subcutaneous treprostinil starting immediately pre-op until
postoperative day #7 to improve immediate postoperative hemodynamics and ultimately
reduce overall length of hospital stay.
The investigators hope to learn how pulmonary vasodilator therapy affects and/or improves
post- operative hemodynamics following the Fontan operation. This knowledge would be very
important in the care of single ventricle patients following the Fontan operation if there
is improvement of immediate outcomes and reduction of hospital length of stay. On a broader
scale, if the use of treprostinil in perioperative Fontan patients can achieve the same
results other centers achieve with the use of a fenestration this may have wide scale
implications in the nationwide treatment of Fontan patients.
- Pediatric patient undergoing Fontan operation and/or pre-Fontan cardiac
catheterization at Lucile Packard Children's Hospital (LPCH)
- Platelet count < 50K (treprostinil can act as a platelet inhibitor and this may place
patient at additional risk of bleeding if already thrombocytopenic)
- Dermatologic condition that renders the patient unable to tolerate a subcutaneous
infusion (can still take part in inhaled vasodilator testing during cardiac
- Currently receiving any vasodilator therapy specifically for the purpose of pulmonary
vsasodilation (phosphodiesterase type 5 inhibitor, endothelia receptor antagonist