Pulmonary Hypertension Association's 2022 International Conference Scientific Sessions Overview.

The considerable progress made in recent years in the diagnosis, risk stratification, and treatment of pulmonary hypertension was highlighted during the most recent edition of the Pulmonary Hypertension Association Scientific Sessions, which was held in Atlanta, Georgia from June 9 to 11, 2022, with the theme: Vision for the PHuture: The Evolving Science and Management of PH. Content presented over the 3-day conference focused on scientific and management updates since the last sessions were held in 2018 and included didactic talks, debates, and roundtable discussions across a broad spectrum of topics related to pulmonary hypertension. This article aims to summarize the key messages from each of the session talks.

Characteristics of Patients With Pulmonary Arterial Hypertension in a Pulmonary Hypertension Association-Accredited Comprehensive Care Center: A Contrast in Features When Compared With US National Registry Data.

Background Since the initial description in the 1980s, our understanding of the diversity of pulmonary arterial hypertension (PAH) has continued to evolve. In this study, we report the characteristics of patients seen in an academic medical center for PAH from August 2020 through November 2021 and contrast those with nationally reported data from the United States Pulmonary Hypertension Scientific Registry (USPHSR).  Study Design Investigators at the University of Utah Pulmonary Hypertension Program prospectively enrolled adult patients diagnosed with WHO Group 1 PAH, who were evaluated between August 2020 and November 2021 in a program-specific registry. Patient exposure and health histories were collected through structured interviews and questionnaires, along with clinical data and medication use. A total of 242 patients were enrolled in the University of Utah Pulmonary Hypertension Registry (UUPHR).  Results Of the 242 enrolled patients, the most common etiology was associated PAH (APAH), accounting for 71.1% of the population. The second largest etiology was idiopathic PAH (IPAH) at 26.4%. The remaining patients were distributed between familial PAH (FPAH), pulmonary veno-occlusive disease (PVOD), and others. Of the total population classified as APAH, 39% of cases were noted as secondary to connective tissue disease (CTD) and 33% as toxin-induced. These represented 28% and 24% of the total population, respectively.  Conclusions In this US-based accredited academic medical center, the etiology of PAH in our patient population contrasts with national registry data. In the UUPHR, APAH, specifically CTD-PAH and toxin-associated PAH, accounts for the majority of patients with PAH. This contrasts with IPAH, which nationally is the most reported cause of PAH. Differences in our population may reflect the regional variation of the referral site, but it is noteworthy for its contrast with historically reported phenotypes.

Management of Pulmonary Arterial Hypertension.

PURPOSE OF REVIEW: This review focuses on the therapeutic management and individualized approach to Group 1 pulmonary arterial hypertension (PAH), utilizing Food and Drug Administration-approved PAH-specific therapies and various interventional and surgical options for PAH. RECENT FINDINGS: The paradigm for the optimal management of PAH has shifted in recent years. Upfront combination therapy with an endothelin receptor antagonist and a phosphodiesterase 5 inhibitor is now widely accepted as standard of care. In addition, there is increasing emphasis on starting prostanoids early in order to delay time to clinical worsening. However, less is known regarding which prostanoid agent to initiate and the optimum time to do so. In order to facilitate shared decision-making, there is an increasing need for decision tools based on guidelines and collective clinical experiences to navigate between pharmacologic and interventional treatments, as well as explore innovative, therapeutic pathways for PAH. SUMMARY: The management of PAH has become increasingly complex. With a growing number of PAH-specific therapies, intimate knowledge of the therapeutics and the potential barriers to adherence are integral to providing optimal care for this high-risk patient population. While current PAH-specific therapies largely mediate their effects through pulmonary vasodilation, ongoing research efforts are focused on ways to disrupt the mechanisms leading to pulmonary vascular remodeling. By targeting aberrations identified in the metabolism and proliferative state of pulmonary vascular cells, novel PAH treatment pathways may be just on the horizon.

Transitioning Between Prostanoid Therapies in Pulmonary Arterial Hypertension.

Background: New oral prostacyclin therapies and prostacyclin agonists have become available for the treatment of pulmonary arterial hypertension (PAH). However, methods for transitioning between oral, inhaled, and parenteral formulations are not well-established, except in the form of case reports and case series. Collectively, these emphasize the lack of a standardized process and approach in transitioning patients between PAH prostanoid therapies. In this case series, we report our experience at an accredited Pulmonary Hypertension center in transitioning between various oral, inhaled, and parenteral prostanoids to offer additional guidance on safe transitions in therapy. Methods: All cases of prostanoid transitions at an accredited Pulmonary Hypertension center from March 2018 to September 2019 were included in this report. The transition approach for each case was developed through a review of the literature, extrapolation of available pharmacokinetic data, and collaboration between pharmacists and clinicians. Results: This case series describes the transition of 3 patients from selexipag to parenteral treprostinil; 1 patient transitioning from parenteral treprostinil to selexipag; 1 patient transitioning from oral treprostinil to parenteral treprostinil; and 1 patient transitioning from inhaled treprostinil to selexipag. Four of the 6 patients presented here were transitioned to an alternate prostanoid on account of clinical worsening, while the remaining 2 patients transitioned due to intolerance of parenteral therapy and poor medication adherence. This case series includes patients with various etiologies of PAH including idiopathic PAH, methamphetamine-associated PAH, and scleroderma-associated PAH. All patients successfully completed each transition without serious adverse events. Conclusions: With the increasing utilization and availability of prostanoids, there is a critical need for a standardized approach in transitioning safely between different formulations without compromising treatment efficacy. In this case series, we present our clinical experiences, guided by available pharmacokinetic data, in transitioning between various prostanoid formulations.