Volume 15: Issue 3

Presentation: A 36-year-old man with known history of pulmonary embolism, deep venous thrombosis, methylenetetrahydrofolate reductase mutation (MTHFR), chronic obstructive pulmonary disease (COPD), and systemic hypertension presented with dyspnea on exertion. He had been diagnosed with Group 4 chronic thromboembolic pulmonary hypertension (CTEPH); therefore, he was referred for consideration of pulmonary thromboendarterectomy (PTEA). Of note, he had an inferior vena cava (IVC) filter placed in 2010. Prior echocardiogram (ECHO) demonstrated severe right atrial (RA) and right ventricular (RV) enlargement and moderate RV systolic hypokinesis. There was an estimated right atrial pressure (RAP) of 15 and a right ventricular systolic pressure

Drug-induced pulmonary arterial hypertension (D-PAH) is a form of World Health Organization Group 1 pulmonary hypertension (PH) characterized by severe small vessel loss and obstructive vasculopathy, which leads to progressive right heart failure and death. To date, 16 different compounds have been associated with D-PAH, including anorexinogens, recreational stimulants, and more recently, several Food and Drug Administration–approved medications. While the clinical manifestations, pathology, and hemodynamic profile of D-PAH are indistinguishable from other forms of PAH, its clinical course can be unpredictable and largely dependent on removal of the offending agent. Since only a subset of individuals develop D-PAH, it is likely that genetic susceptibility plays a role in pathogenesis, but characterization of the genetic factors responsible for disease susceptibility remains incomplete. Besides aggressive treatment with PH-specific therapies, the major challenge in the management of D-PAH remains the early identification of compounds capable of injuring the pulmonary circulation in susceptible individuals. Institution of pharmacovigilance, precision medicine strategies, and global warning systems will help facilitate identification of high-risk drugs and institute regulation strategies to prevent further outbreaks of D-PAH.

Human immunodeficiency virus (HIV) infection is a well-recognized cause of pulmonary arterial hypertension (PAH). Worldwide, an estimated 37 million individuals are infected with HIV, and the availability of effective HIV therapy means that more of these patients are living longer and developing cardiovascular complications, including PAH. In European populations, it is estimated that 0.5% of patients with HIV infection develop moderate to severe PAH, so that HIV infection may be one of the most common causes of PAH worldwide. Untreated, the prognosis of HIV-associated PAH is particularly poor, and a large proportion of patients with HIV infection live in resource-poor regions of the world where PAH therapy is not available. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for HIV-associated PAH are summarized.

Schistosomiasis is the most common parasitic disease associated with pulmonary arterial hypertension (PAH). It induces remodeling via complex inflammatory processes produced by the parasite eggs. Changes in the pulmonary vasculature after Schistosoma infection are common, but may not always be associated with a clinical manifestation of PAH. Those patients who presented with PAH show clinical signs and symptoms that are not distinguishable from other forms of PAH.

The Pulmonary Hypertension Association's 10^th International Conference occurred June 17 through 19, 2016 in Dallas, Texas. 1,028 patients and caregivers attended the 3-day meeting, and 447 healthcare professionals participated in scientific sessions, abstract presentations, and research opportunities in addition to speaking at patient- and family-focused programs. Advances Guest Editor and Conference Scientific Chair Tim Lahm, MD, along with David Badesch, MD, Professor of Medicine and Clinical Director, Pulmonary Hypertension Center, University of Colorado Health Sciences Center; Kara Goss, MD, Assistant Professor of Medicine, Division of Pulmonary and Critical Care, Department of Medicine, University of Wisconsin School

Editor's Memo

Guest Editors' Memo

PH Grand Rounds: Chronic Thromboembolic Disease Cloaking Unilateral Pulmonary Artery Hypoplasia

Drug-Induced Pulmonary Hypertension: The First 50 Years

HIV-Associated Pulmonary Hypertension: A Global Perspective

Pulmonary Vascular Diseases Secondary to Schistosomiasis

High-Altitude Pulmonary Vascular Diseases

Roundtable: Reflections on PHA's International Conference

Conference Abstracts

PH Professional Network: Conference 2016 Recap: 25 Years of Progress

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease