A Study to Assess the Safety and Efficacy of Treprostinil to Facilitate Liver Transplantation in Patients With Portopulmonary Hypertension
A Study to Assess the Safety and Efficacy of Treprostinil to Facilitate Liver Transplantation in Patients With Portopulmonary Hypertension
This was a multicenter, prospective, observational, open-label study. Patients meeting
 inclusion/exclusion criteria received treatment with treprostinil as recommended by their
 treating physicians and were followed according to standard of care. This observational study
 collected clinical data and biologic specimens from patients who were treated for
 portopulmonary hypertension (PoPH), with a goal of achieving hemodynamic parameters
 appropriate for orthotopic liver transplantation candidacy, including mean pulmonary arterial
 pressure (mPAP) less than 35 mmHg and pulmonary vascular resistance (PVR) less than 3
 Wood-units (WU) at Week 24 in patients with severe PoPH.
Treprostinil is approved as a continuous subcutaneous (SC) or intravenous (IV) infusion by
 the FDA for the treatment of WHO group I PAH with New York Heart Association (NYHA)
 Functional Class II, III or IV symptomatology. To date, treprostinil has not been studied in
 the setting of PoPH; however, it is commonly prescribed in this setting. This was an
 observational, open-label, multicenter study which documented the safety and efficacy profile
 of this agent in PoPH to facilitate orthotopic liver transplantation (OLT).
Inclusion Criteria:

 -  Patients must:

 1. Had portal hypertension.

 2. Be otherwise suitable candidates for OLT.

 3. Had severe pulmonary arterial hypertension (PAH) defined as a resting mean
 pulmonary arterial pressure (mPAP) >35 mmHg and pulmonary vascular resistance
 (PVR) ≥3 Wood Units (WU) by right heart catheterization (RHC) performed as part
 of standard of care evaluation within 90 days of enrollment.

 4. Treprostinil therapy must be recommended by the treating physician per standard
 of care.

 5. Be NYHA Functional Class II, III, or IV.

 6. Had pulmonary capillary wedge (PCW) pressure ≤18 mmHg and transpulmonary gradient
 (TPG) ≥15 mmHg.

 Exclusion Criteria:

 -  Patients must not:

 1. Had received any any investigational therapy as part of a clinical trial for any
 indication within 30 days prior to enrollment.

 2. Had a change in dose of treatment for PAH (bosentan [Tracleer], ambrisentan
 [Letairis], tadalafil [Adcirca], or sildenafil [Revatio]), within 30 days prior
 to enrollment. That is, subjects may have been treated with any of these agents
 provided the dose was stable for at least 30 days prior to enrollment.

 3. Had renal failure requiring hemodialysis.