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Advances in PH Journal

Refractory Pulmonary Hypertension, 2016 (Vol 15, No 1)
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  • Refractory Pulmonary Hypertension, 2016 (Vol 15, No 1): Table of Contents

  • Editor's Memo
    Patients with pulmonary arterial hypertension (PAH) refractory to conventional pharmaceutical intervention with PAH-specific medications remain a significant challenge to providers.
  • Guest Editor's Memo
    In this issue of Advances, we address the options for the failing patient; hopefully, the following articles will better prepare you for this situation and will provide you with a blueprint of how to approach such a patient.
  • Navigating the Road to Transplant in Pulmonary Arterial Hypertension: A Road Less Taken
    Dramatic advances in therapy for pulmonary arterial hypertension (PAH) in the last 20 years have improved survival from a median of 2.5 years in the pretreatment era to 7.5 years currently. However, impressive as that may seem, it is important to note that a median survival of 7.5 years is equivalent to that of surgically resected non-small cell lung cancer, thus underscoring the importance of lung transplantation as a treatment option in patients with PAH. In this edition of Advances, Edelman has reviewed the pathway to transplantation for patients with PAH, detailing the recommendations for timing of referral, listing for lung transplantation, the role of the lung allocation score in allocating a donor organ, and the outcome of lung transplantation.
  • Navigating the Road to Transplantation for Pulmonary Arterial Hypertension
    Transplantation is an important component of treatment for pulmonary arterial hypertension (PAH) patients with severe limitations despite optimal medical therapy. Although the risks of transplantation remain significant, appropriate candidates may achieve excellent functional outcomes and long-term survival. As PAH therapy and transplantation have advanced, the approaches to transplant referral and listing have changed. This article will review criteria and thresholds for transplant referral and listing, novel approaches to expanding the pool of donor organs, options for bridging to transplantation, and the ways in which the current system for organ allocation have evolved. Knowledge of these concepts will help to ensure timely referral and navigation of the course to successful transplantation for PAH patients.
  • Balloon Dilation Atrial Septostomy and Potts Anastomosis for Severe Pulmonary Arterial Hypertension: Why, When, and How
    Despite advances in pharmacologic treatment, pulmonary arterial hypertension (PAH) remains a fatal disease. In recent years, surgical/interventional approaches including balloon dilation atrial septostomy and Potts anastomosis have been applied to improve the hemodynamic variables associated with right ventricular failure in the setting of PAH. These interventions may improve quality of life and prolong survival in this population. In this review, we will discuss the role of these 2 therapeutic alternatives in the management of PAH.
  • Pulmonary Hypertension and Palliative Care: What, When, Where, and Why?
    Communication with patients who have PH can be delicate and requires an understanding of the disease’s process, trajectory, and prognosis. PC teams possess communication skills that may benefit patients and providers with QOL optimization, delivery of difficult news, advanced care planning, and shared decision-making.
  • PHPN: Pulmonary Hypertension Patient Navigation: Avoiding the Perfect Storm
    Pulmonary arterial hypertension (PAH) is a progressive, incurable disease that presents a challenging journey for all involved. Specialized, complex care and treatment is needed for this population of patients, and should be provided in an organized, systematic manner to promote optimal patient outcomes. The concept of patient navigation can be used as a framework for the pulmonary hypertension (PH) center, so that care delivery is well structured and PAH patients have a guide to assist them through all aspects of the health care continuum. This article will focus on how a PH patient navigation program can be implemented and the role of a PH nurse navigator, using the Christiana Care Health System Pulmonary Hypertension Program in Newark, Delaware, as an example. There have been many advances in PAH diagnosis and treatment in the last 20 years, and the time has come to introduce a PH patient navigation model that can be used as a guide to structure PH programs.
  • What Strategies Might Reduce Barriers to Routine Integration of Palliative Care Services in Patients With Pulmonary Arterial Hypertension?
    The discussion that follows considers some practical steps that might be taken in the near term to lower the barriers to palliative care access in patients with PAH, such that the benefits described so well by Fenstad et al. may be better realized. These barriers to improved integration of palliative care with PAH may be loosely classified under 3 headings: those that can be addressed by increased education, those that can improve patient and provider communication, and those that can address access to palliative care services.
  • Pulmonary Arterial Hypertension: “A Journey to Lung Transplant”
    A 31-year-old female presented to her family physician 15 years ago with subtle but gradually worsening dyspnea on exertion (DOE) over the previous 5 years. Prior to these symptoms, she had been very active, running 5 miles a day. She felt her running was now limited by this progressive shortness of breath (SOB).
  • Dealing With End-Stage Pulmonary Arterial Hypertension
    Guest editor and editor-in-chief-elect Hap Farber, MD, led an insightful–and lively– discussion among an international group of practitioners to share their opinions and expertise on the state of the art in managing end-stage pulmonary hypertension. The discussants are Drs. Olivier Sitbon, Professor of Respiratory Medicine at the South Paris University, France; Maria Crespo, associate medical director of the lung transplant program at the University of Pittsburgh; and Adaani Frost, professor at the Institute of Academic Medicine, and director of the Houston Methodist Lung Center.