Perioperative Treprostinil in Pediatric Patients Undergoing the Fontan Operation

• Status: Completed
• Trial end date: 2020-12-01
• Target enrollment: 0
• Participant gender: All

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.

• Phase: N/A
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Stanford University
• Treatments: Treprostinil

Criteria

Inclusion Criteria:

• Pediatric patient undergoing Fontan operation and/or pre-Fontan cardiac catheterization at Lucile Packard Children's Hospital (LPCH)

Exclusion Criteria:

• 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 catheterization)

• Currently receiving any vasodilator therapy specifically for the purpose of pulmonary vsasodilation (phosphodiesterase type 5 inhibitor, endothelia receptor antagonist and/or prostacyclin).