Real-world experiences and treatment patterns among congenital heart disease patients with associated pulmonary vascular disease: Results from a real-world survey in the United States.

Background: Limited data exist on the use of drugs in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). Therefore, we evaluated their real-world patient journey, including symptomatology, diagnostic pathway, treatment patterns, and guideline adherence.

Methods: Data were drawn from the Adelphi Real World pulmonary hypertension congenital heart disease Disease Specific Programme (DSP), a cross-sectional survey of clinicians and patients with PAH-CHD or Fontan circulation associated with elevated pulmonary vascular resistance (PVR).

Characteristics of Patients with Pulmonary Arterial Hypertension Receiving Selexipag in the SPHERE Registry by Race and Ethnicity.

Introduction: Racial/ethnic minority populations in the US have a high burden of pulmonary arterial hypertension (PAH).

Objective: To evaluate demographics, disease characteristics, prescribing patterns, hospitalization, and survival in racial/ethnic groups in the SPHERE registry.

Methods: SPHERE was a US, multicenter, prospective, observational registry of adults prescribed selexipag in clinical practice (November 2016-March 2020).

The Role of Telehealth in PAH Management: Benefits, Barriers, and Solutions.

Telehealth utilization increased during the coronavirus disease-2019 (COVID-19) pandemic, which encouraged remote patient management. The identification of optimal strategies to enhance telehealth and address barriers to its use for patients with pulmonary arterial hypertension (PAH) may improve patient outcomes. Physicians ( = 11) and advanced practice providers ( = 6) based in the United States with experience with telehealth in PAH were recruited to a double-blinded modified Delphi panel (two survey rounds and a virtual consensus meeting).

Changes in REVEAL Lite 2 risk status are associated with long-term outcomes in patients with pulmonary arterial hypertension: A post-hoc analysis of the GRIPHON study.

Background: Mortality risk assessment informs clinical management of pulmonary arterial hypertension (PAH). The Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) Lite 2 is a simplified risk calculator discriminating 1-year mortality risk.

Methods: This post-hoc analysis of the phase 3 GRIPHON study assessed changes in REVEAL Lite 2 risk score with selexipag versus placebo and whether changes were prognostic or predictive of time to first morbidity/mortality (M/M) event.

Oral Prostacyclin Pathway Agents Used in PAH: A Targeted Literature Review.

Purpose: Pulmonary arterial hypertension (PAH) is a rare and progressive pulmonary vascular disease that can result in right heart failure and death. Oral prostacyclins play an important role in the management of intermediate-low risk PAH. This targeted literature review (TLR) aimed to identify and compare evidence supporting use of oral prostacyclin pathway agents (PPAs: selexipag and oral treprostinil) in intermediate-low risk PAH.

Factors associated with discontinuation of treatment for pulmonary arterial hypertension in the United States.

Information on factors leading to pulmonary arterial hypertension (PAH) treatment discontinuation is limited. This study analyzed 12,902 new PAH medication users to identify predictors of treatment discontinuation. Treatment by accredited pulmonary hypertension centers and combination therapy with PAH agents from different classes were less likely to result in discontinuation.