Volume 13: Issue 2

Section editor's note: Welcome to a new section in Advances titled “Pulmonary Hypertension Grand Rounds” (PHGR), which we are introducing in this issue. With this addition, Advances offers a unique opportunity for trainees and junior faculty interested in pulmonary hypertension (PH). This section will highlight cases with literature reviews written by young clinicians and researchers in PH. PHGR was created to use patient cases to integrate individual clinical expertise with the best available evidence from the literature to enable the best decisions for patients. As with the rest of the journal, our audience continues to be physicians, researchers, nurses, students, residents, and fellows interested in both the care of individual patients and the advances in our field. We welcome comments about the section and inquiries regarding submitting a case for publication. Please direct inquiries to advancesgrandrounds@PHAssociation.org

In 2013, the US Food and Drug Administration (FDA) approved 3 new oral drugs for the treatment of pulmonary arterial hypertension (PAH; World Health Organization [WHO] Group 1 pulmonary hypertension [PH]). These include the endothelin receptor antagonist macitentan, the soluble guanylate cyclase stimulator riociguat, and the prostacyclin analogue treprostinil. In addition, riociguat was approved for the treatment of patients with inoperable or postsurgery recurrent or persistent chronic thromboembolic PH (CTEPH; WHO Group 4 PH). The approval of these drugs has several important clinical implications: first, in a disease where many of the currently available treatments are complicated by significant side effects and/or complex administration regimens, the availability of new oral drugs clearly represents a valuable addition to the armamentarium. Second, the macitentan study was the first long-term, event-driven trial to be published in the PAH field, making the results more robust and paving the way for improved clinical trial design in the future. Third, riociguat is the first FDA-approved medical treatment regimen for selected CTEPH patients, thus providing a critical treatment option for patients with inoperable or recurrent/persistent CTEPH. Lastly, the approval of oral treprostinil made this drug the first oral prostacyclin analogue to be available in the United States. In this article, the authors will discuss the mechanisms of action of macitentan, riociguat, and oral treprostinil; review the landmark trials that led to the FDA approval of these drugs, and discuss their clinical use.

Research in pulmonary hypertension (PH) in general and in pulmonary arterial hypertension (PAH) in particular has been extremely active in the past few years. The current therapeutic landscape includes multiple compounds shown to be active in modulating and ameliorating the pathological changes in the endothelin, nitric oxide (NO), and prostacyclin pathways. In addition, development of new compounds targeting pathways of pulmonary vascular remodeling due to inflammation, fibrosis, and oxidative stress is underway. This review summarizes the most recent achievements and newly conducted clinical trials—some with novel trial design—for treatment of PH and PAH.

The Institute of Medicine (IOM) monograph titled, Crossing the Quality Chasm: A New Health System for the 21st Century, recognized the importance of communication in health care. The report identified patient-centered care, described as “care that is respectful of and responsive to individual patient preferences, needs, values, and ensuring that patient's values guide all clinical decisions,” as one of the 6 dimensions that define quality in health care.1 Through active listening, empathy, and negotiating the health care provider contributes to patient-centered care.

Patient-centered care is essential to providing excellent treatment to patients diagnosed with pulmonary arterial

Memos

Editor's Memo

PH Grand Rounds: Puzzling Etiology of Pulmonary Hypertension Resolved

New Oral Drugs for Pulmonary Arterial Hypertension: Macitentan, Riociguat, and Treprostinil

Future Therapies in PAH: Update on the New Landscape and Drugs in the Pipeline

Ambulatory Hemodynamic Monitoring in the Management of Pulmonary Arterial Hypertension

Roundtable: New Treatments, New Challenges for Pulmonary Hypertension

Ask the Expert: Are Newer Management Approaches Increasing the Economic Burden of Treating Pulmonary Arterial Hypertension?

Pulmonary Hypertension Care Centers: Role of the Review Committee

PHPN: Keeping the Patient at the Forefront

Racial and Ethnic Equity in Pulmonary Arterial Hypertension Clinical Trials

Multiomics Integration for Identifying Treatment Targets, Drug Development, and Diagnostic Designs in PAH

Tyrosine Kinase Inhibitors for Treatment of Pulmonary Arterial Hypertension

Full Issue PDF

Editor’s Memo