Program

Patients with pulmonary hypertension who become pregnant face an increasingly complex situation, both pathophysiologically and socially. Traditionally, becoming pregnant, while living with pulmonary arterial hypertension has been avoided and PAH patients who are pregnant are counseled on termination. Through experience, we now know that some PAH patients can proceed through pregnancy and delivery. However, this process must be accompanied by close monitoring from a multidisciplinary team, frank discussion of associated risks and anticipatory planning for the possibility of life support. With a unique patient population and little data to guide medical decision making, each patient's case must be approached thoughtfully and uniquely. In this discussion, speakers will outline the pathophysiologic changes associated with pregnancy; their institutional approach to assessment and monitoring through pregnancy and delivery; and the psychosocial, financial and increasing legal issues faced by patients in this high-risk condition.

Learning objectives: At the end of this session, participants should be able to:

• Discuss the complex changes in physiology associated with pregnancy and PAH, and how these are followed with monitoring.
• Review the nuances of pregnancy monitoring and delivery options for patients with PAH, including the potential for extracorporeal membrane oxygenation and expedited transplantation.
• Develop a deeper appreciation for the financial, legal and psychosocial issues faced by patients with PAH who become pregnant.
• Discuss the gaps in research and knowledge of management of patients with PAH and pregnancy, as well as potential opportunities for trainees.

Speakers: Yuri Matusov, MD, FACP, ATSF; Mariam Naqvi, MD; Inderpreet Saini, NP-BC, MSN

Despite pulmonary arterial hypertension-specific drugs, PAH still exhibits poor median survival, persistent occlusive pulmonary arteriopathy and right ventricle dysfunction or failure. This session will provide an overview of advances in stem and progenitor cell adjunctive therapies to address unmet treatment needs through a "bed to bedside journey." Topics discussed in this session include preclinical data in rat models of PAH and the recently published ALPHA Phase 1 trial in patients with PAH. The session will also provide the audience with exciting new cellular data and approaches geared to meet the needs of current PAH management.

Learning objectives: At the end of this session, participants should be able to:

• Discuss the unmet treatment needs regarding current PAH therapies geared towards PAH and progressive RV dysfunction.
• Summarize new insights into the upcoming field of regenerative medicine, which describes cell and/or gene therapies to meet conditions with unmet medical needs.
• Review how cellular therapies could meaningfully impact the pathobiological aberrations in both the pulmonary vessels and the right ventricle, as key adjunctive therapy options, to improve PAH outcome and patient well-being.

Speakers: Michael Lewis, MD

This session will feature a return of the fan-favorite session from the 2019 PHPN Symposium based on the gameshow JEOPARDY! The session will include an emcee, three contestants and categories across the spectrum of pulmonary arterial hypertension practice including practical management issues, diagnosis, medications and patient issues. Attend the session to fact-check contestant responses and join the discussion between the emcee and contestants.

Learning objectives: At the end of this session, participants should be able to:

• Identify common elements of the diagnostic workup of patients with PAH, chronic thromboembolic pulmonary hypertension or PH-related interstitial lung disease.
• Review common elements of the day-to-day care of patients with PAH, CTEPH or PH-ILD.
• Discuss common elements of the treatment of patients with PAH, CTEPH or PH-ILD including medications, complications and practical issues of management. Survey areas of the pathophysiology of PAH.

Speakers: Robert Schilz, DO, PhD; Jennalyn Mayeux, DNP, APRN, NP-C; Denise Lewis, RN, BSN; Johnell Diawn, RN, BSN

Safe and effective delivery of subcutaneous treprostinil in the inpatient setting is essential for a successful pediatric pulmonary hypertension program. Maintaining bedside nursing skills, medication protocols and hospital supplies for highly specialized medications and pumps is a difficult aspect of a PH program and part of what defines a program of excellence. Pediatric PH programs have the added challenge to overcome the nuances of delivering subcutaneous treprostinil therapy to the smallest patients, including neonates. The changing landscape of subcutaneous treprostinil delivery systems across the U.S. is disrupting the ability to deliver subcutaneous treprostinil in the inpatient setting. With new pumps coming onto the market and older pumps fading out, PH programs and their affiliated hospitals are at a crossroads.

In this session, presenters from three pediatric PH centers that have adopted the Medfusion pump will outline best practices for the delivery of subcutaneous treprostinil to the hospitalized pediatric PH patient. Speakers will review options of pumps available for use, administrative hurdles associated with obtaining the pumps, pearls for Medfusion pump delivery of subcutaneous Remodulin, hands-on practice using the pump and examples of inpatient training resources.

Learning objectives: At the end of this session, participants should be able to:

• Distinguish between the pros and cons of available pump options for inpatient subcutaneous treprostinil delivery for the pediatric PH patient.
• Review the hospital procurement process and administrative hurdles of obtaining a new type of pump.
• Discuss developed protocols and training resources for using the Medfusion pump to safely delivery subcutaneous treprostinil in the inpatient setting.

Speakers: Anna Brown, DNP, MSN, CPNP-PC; Emma Jackson, ARNP, MSN, PNP; Jess MacLean, MSN, FNP-BC; Kelly Merrill, RN

In this session, speakers will present sample cases to highlight how a multidisciplinary care team would select treatments for patients with chronic thromboembolic pulmonary hypertension. Speakers will review the cases and participate in an interactive discussion on how to handle each case. Audience members will have the chance to test their knowledge and learn practical decision-making skills on when to refer for surgical interventions.

Learning objectives: At the end of this session, participants should be able to:

• Discuss the importance of a multidisciplinary approach to CTEPH care and post-CTEPH care.
• Recognize the need for careful diagnostic phenotyping using precise imaging and clinical expertise.
• Compare the use of balloon pulmonary angioplasty versus pulmonary thromboendarterectomy for CTEPH treatment.
• Identify the appropriate use of medical therapy in pulmonary hypertension.
• Explain the appropriate patient selection for different modalities.

Speakers: Stephen Chiu, MD; Vladimir Lakhter, DO; Sonja Bartolome, MD; Micah Fisher, MD

The University of California San Francisco Pulmonary Hypertension Program has consistently embraced a holistic approach to caring for individuals with methamphetamine-induced PAH. Practitioners at the center believe that acknowledging and managing pulmonary arterial hypertension in a supportive environment can serve as a crucial motivator for patients to alter their drug use patterns or achieve complete cessation, ultimately empowering patients on their journey towards recovery and improved overall health. In this session, UCSF representatives will share experiences and best practices in optimizing care for this vulnerable population. In addition, speakers will highlight pilot programs that incorporate the use of addiction medicine expertise. The session aims to showcase how efforts of the UCSF PH Program raise awareness about the disparities these patients encounter due to varying health care perspectives.

Learning objectives: At the end of this session, participants should be able to:

• Review the physiological and psychological effects of methamphetamine abuse, with a particular focus on its association with pulmonary arterial hypertension.
• Summarize the importance of addressing substance use disorder alongside PAH treatment, examining integrated care models that combine medical, psychological and social support services.
• Apply holistic care approaches that emphasize the importance of a multidisciplinary team in providing comprehensive care for this vulnerable population, aiming to promote recovery and long-term health improvements.
• Discuss new and innovative programs including pilot initiatives designed to improve outcomes for patients with meth-induced PAH.

Speakers: Sara Bravo, MSN, RN, AGCNS-BC, PHN; Nicholas Kolaitis, MD; Anna Deering, MSW, LCSW

In this session, presenters will share the current landscape of artificial intelligence in treating pulmonary hypertension including, early diagnosis, classification and use of prediction models to aid in prognostication. Speakers will discuss published studies of AI applied to various datasets and tests to help PH diagnosis and classification. In addition, speakers will share keys to the successful development of AI tools in PH and limitations of the use of AI in PH, including those related to interpretability and implementation. The session will provide a framework and tips to direct future studies and development of robust AI tools to help improve outcomes in PH patients.

Learning objectives: At the end of this session, participants should be able to:

• Discuss the role of artificial intelligence in pulmonary hypertension.
• Review the various applications of AI to help in the diagnosis, classification, risk stratification and prognostication of PH patients.
• Interpret the current literature on AI in PH and discuss the limitations of AI tools.
• Summarize the future directions for AI research and PH.

Speakers: Akshay Kohli, MBBS, MD; Anjali Vaidya, MD, FACC, FASE, FACP; Hilary DuBrock, MD

During this symposium research highlight, authors of the highest-scoring abstracts will present their works in quick succession. Featured topics include managing pregnancy in pulmonary arterial hypertension, remote monitoring, medication transitions and new therapies. There will be time for questions. Authors will also be available throughout the symposium for additional discussions.

Sepsis is the leading cause of intensive care unit-related death worldwide and is particularly challenging in patients with pulmonary arterial hypertension and right ventricular dysfunction. Successful management of patients with pulmonary hypertension and sepsis begins with treatment of the underlying cause of sepsis and requires an understanding of the complex interplay of factors that can lead to right ventricular decompensation. This session will review the pathophysiology of sepsis as it affects the myocardium and its function. The presenter will outline specific measures that serve to preserve optimal right ventricular function in patients with PH and septic shock, including a review of tools for managing sepsis in PH and the roles of pulmonary vasodilator and systemic vasoactive therapies.

Learning objectives: At the end of this session, participants should be able to:

• Review the pathophysiology of septic cardiomyopathy.
• Discuss contrasting structural and functional characteristics of right and left ventricles with emphasis on right ventricular function in PAH.
• Summarize the interplay of factors leading to right ventricular decompensation in the PH patient with septic shock.
• Discuss tools to assess and maintain optimal right ventricular function in the septic patient with PH and right ventricular compromise.
• Review the roles of pulmonary and systemic vasoactive therapies in managing septic shock in patients with severe PH.

Speakers: Kunal Gada, MBBS, MD

This session will highlight creative, multidisciplinary approaches to improving quality of life in patients with pulmonary arterial hypertension. Speakers will discuss early involvement of palliative medicine; the positive impacts of joining a support group; and advancing patient outcomes through improving mental health. Presenters will also review tips for patients managing a "hidden disease" including self-advocacy, how to conserve energy, returning to work and water-based activities for patients on subcutaneous therapies. Pediatric pulmonary hypertension considerations including engaging with activities, school and friends will be discussed.

Learning objectives: At the end of this session, participants should be able to:

• Describe innovative ways to make life with PAH easier.
• Identify resources that empower patients with PAH to re-engage in activities that bring joy and fulfillment.
• Identify benefits of getting involved in a support group.
• Describe strategies for improving mental health.
• Describe benefits of involving palliative medicine early in the diagnosis and treatment plan.

Speakers: Debbie Kittel, BSN, RN, CCRN; Julie Huston, PhD; Jennica Johns, MD; Tiffani Brown; Scott Visovatti, MD; Allyson Rupp, MSW, LCSW, ACM-SW

The recent addition of the activin signaling inhibitor therapy as the fourth agent to treat pulmonary arterial hypertension WHO Group 1 provides an opportunity for a de-escalation of an existing PAH therapy for the health care team and patient. During this session, panelists will share their experiences of de-escalating prostacyclin therapies.

Learning objectives: At the end of this session, participants should be able to:

• Review the four treatment pathways available for pulmonary arterial hypertension WHO Group 1 patients.
• Summarize clinical and hemodynamic parameters available to initiate and guide the de-escalation process of prostacyclin therapy.
• Discuss the institutional experiences of de-escalating a prostacyclin.

Speakers: Ai Jin Lee, MSN, RN, AGCNS-BC, CCRN; Charlotte Lipsky, MSN, NP; Sara Bravo, RN, AGCNS-BC; Sheryl Wu, PharmD, APh, BCPS

Cardiopulmonary exercise testing is gaining clinical acceptance due to its ability to quantify patient effort, aerobic capacity and ventilation/profusion coupling within the pulmonary system. In this session, the speaker will explain the hallmark abnormalities noted on cardiopulmonary exercise testing in patients with pulmonary hypertension and how to use this information to better understand the mechanisms of exercise intolerance. Application and measurement techniques will be discussed, highlighting the personnel, equipment and backgrounds needed to perform cardiopulmonary exercise testing. Reports on patients with known pulmonary hypertension will be explained with in-depth analysis of the Wasserman 9-panel plot. The session will conclude with insights into prognosis determinants and how cardiopulmonary exercise testing reflects the response to therapeutic interventions.

Learning objectives: At the end of this session, participants should be able to:

• Identify the mechanisms and ventilatory consequences of the anaerobic threshold.
• Review the mechanisms by which impaired pulmonary perfusion affects ventilatory efficiency.
• Summarize how impaired oxygen delivery and ventilatory inefficiency collectively impact exercise tolerance.
• Discuss CPET markers and the cutoff values that serve as prognosticators for PH.

Speakers: Zachary Fulkerson, MD, PhD

This session will consist of a series of lectures by subspecialists on topics seminal to developmental lung disease. Speakers will review pediatric pulmonary hypertension, echocardiographic findings; diagnosis and assessment, including genetics and pathology; and therapies, including interventional treatments. At the end of the session, speakers will take questions from the audience and lead an interactive, case-based discussion.

Learning objectives: At the end of this session, participants should be able to:

• Review atypical course of persistent pulmonary hypertension of the newborn and possible etiologies.
• Discuss how to incorporate new and emerging treatment options when establishing goals of medical therapy for pulmonary arterial hypertension patients.
• Discuss limitations of imaging and the role of cardiac catheterization for diagnosis of pediatric PH patients.
• Differentiate timing for diagnostic testing and application of different testing modailities.
• Summarize the need for mutidisciplinary collaboration in follow up of patients with developmental lung disease.

Speakers: Nidhy Varghese, MD; Roberta Keller, MD; Kinjal Parikh, MD

This session is designed for pulmonary hypertension-treating health care professionals to enhance their understanding of subcutaneous medication delivery systems. In this session, speakers will cover device selection, administration techniques, troubleshooting common complications and patient education strategies. Participants will gain practical knowledge on optimizing subcutaneous therapy to improve patient outcomes and adherence.

Learning objectives: At the end of this session, participants should be able to:

• Describe the pharmacology and clinical indications for subcutaneously administered prostacyclin analogs.
• Identify best practices for pump setup, infusion site selection and patient training to enhance adherence and minimize complications.
• Recognize and manage adverse reactions, device malfunctions and patient-reported concerns to ensure therapy continuity.

This activity is supported by an educational grant from United Therapeutics.