A Registry for Patients Taking Uptravi

Conditions

Pulmonary Arterial Hypertension

Brief summary

This is a US multi-center, prospective, real world, observational drug registry enrolling patients actively treated with Uptravi. Participating patients will be followed prospectively for a maximum of 18 months from the date of enrollment into the registry.

Escribano Subias P, Chakinala MM, Chin KM, Didden EM, Doad G, Farber HW, Gaine S, Hemnes AR, Highland KB, Lange TJ, McLaughlin V, Muller A, Rahman M, Remeňová T, Söderberg S, Venkatesh A, Kim NH.

2026-01-12

10.1007/s40801-025-00536-4

Disease characteristics, treatments, and outcomes of patients with pulmonary arterial hypertension treated with selexipag in real-world settings from the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry).

McLaughlin V, Farber HW, Highland KB, Hemnes AR, Chakinala MM, Chin KM, Han M, Cho M, Tobore T, Rahman M, Kim NH.

2023-09-2912 citations

10.1016/j.healun.2023.09.016

Patient and disease characteristics of the first 500 patients with pulmonary arterial hypertension treated with selexipag in real-world settings from SPHERE.

Kim NH, Hemnes AR, Chakinala MM, Highland KB, Chin KM, McLaughlin V, Zhao C, Narayan V, Farber HW.

2021-01-1512 citations

10.1016/j.healun.2021.01.006

Interventions

None listed

Study design

Observational model

OTHER

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

18 Years to 99 Years

Healthy volunteers

No

Inclusion criteria

* Signed patient informed consent form (ICF). * Patients ≥ 18 years of age at time of Uptravi initiation, and * Patients who initiate Uptravi: * at enrollment, or * less than or equal to 60 days prior to enrollment and have a documented titration regimen (defined as all documented dose changes including, but not limited to: starting dose and dates and highest tolerated dose and dates)

Exclusion criteria

* Patients previously exposed to Uptravi treatment during a clinical trial. * Patients that previously discontinued Uptravi for any reason, prior to study enrollment (discontinuation defined as an interruption of therapy greater than or equal to 30 days). * Patients enrolled in a blinded clinical trial or in a clinical trial involving an unapproved drug.

Design outcomes

Primary

Measure	Time frame	Description
Using Electronic Medical Records, the change in Hemodynamics (by Right Heart Catherization), WHO FC and 6 Minute Walk Distance (by meters) from diagnosis to registry enrollment and to EOS will be measured/collected.	Followed prospectively for a maximum of 18 months from the date of enrollment into the registry.	To describe demographics and disease characteristics of patients treated with Uptravi.
The initial dose (in micrograms), dose at selected intervals (in days), highest dose, maintenance dose, time between titrations, time from initiation to maintenance dose (in days) and total duration of exposure to Uptravi (in days) will be collected.	Followed prospectively for a maximum of 18 months from the date of enrollment into the registry.	To describe the dosing regimens and titration of Uptravi, including any transition from other PAH specific therapies to Uptravi and from Uptravi to other prostanoids.
Occurrence of AEs and SAEs (incl. hospitalizations), discontinuation of Uptravi and reason for stopping and occurrence of all-cause death by relationship to PAH.	Followed prospectively for a maximum of 18 months from the date of enrollment into the registry.	To describe the clinical course of patients treated with Uptravi.

Countries

Puerto Rico, United States

Outcome results