Volume 12: Issue 3

The Clinical Trials Update highlights new and ongoing research trials that are evaluating therapies for PAH. In this issue, Fernando Torres, MD, examines a study on patients with pulmonary hypertension associated with left ventricular systolic dysfunction.

Patients with chronic obstructive pulmonary disease (COPD) often present with mild pulmonary hypertension (PH). This finding has been attributed to hypoxic pulmonary vasoconstriction. However, a small proportion of COPD patients will present with moderate or severe elevations in their pulmonary artery pressure (PAP), and these patients appear to have worsened symptoms and survival when compared to patients with milder elevations in PAP. The diagnosis of PH in COPD may be difficult, due to inaccuracies in the echocardiographic estimates of PAP in these patients. Additionally, many patients with COPD will also have comorbid conditions such as diastolic heart failure, systolic heart failure, or obstructive sleep apnea, which may cause increased pulmonary pressures through other mechanisms. Clinical trials investigating the effect of PH-specific therapy for patients with PH and COPD have been small, with mixed results. A careful evaluation for other causes of PH and hemodynamic evaluation will help guide medical therapy for this group of patients.

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with a variable natural history. Pulmonary hypertension (PH) is frequently found in patients with IPF and is associated with an almost 3-fold increase in the risk of death. Pulmonary hypoxic vasoconstriction plays an important role in the pathogenesis of PH in IPF (PH-IPF), although it has become clear that it is not the only mechanism involved. While invasive right heart catheterization is the gold standard modality of hemodynamic assessment, there has been increasing interest in noninvasive testing, such as Doppler echocardiogram, as complementary methods of assessing right ventricular function in these patients. While the expanding array of pharmacologic options for the treatment of pulmonary arterial hypertension has engendered increased interest in the application of these therapies for PH-IPF, supportive evidence for benefit is lacking.

Many caregivers are regularly challenged with questions regarding portable oxygen. There are more options available than ever and one would assume that would simplify things. However, there is more mainstream marketing directed at patients, and some systems are simply not sufficient for patients' needs.

LOOKING BACK

Forty years ago the only existing “home oxygen” option available was when an industrial supplier of medical gases would deliver a series of “H” cylinders (with brass regulators) to a patient's home. Times have changed. Today's choices include home oxygen concentrators, portable oxygen concentrators (POCs), aluminum portable (lighter and smaller) tanks, oxygen conserver (demand)

PHA Classroom

PHA Classroom hosts and archives live e-learning events targeted toward patients and caregivers that cover a wide array of topics to help improve their lives. Recent recordings include:

Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease
Surviving Survivor's Guilt
Chronic Thromboembolic Pulmonary Hypertension—What You Need to Know
Exercise-Induced Pulmonary Hypertension—What Is It and What Do We Do About It?

You and your patients can view recent recordings and upcoming live e-learning events at www.PHAssociation.org/Classroom.

PHA Resources for Patients

PHA offers ongoing education and information for patients and caregivers at every stage of their PH journeys. We

Memos

Editor's Memo

Ask the Expert: When Is Testing Beyond Overnight Oximetry Indicated for Assessment of Sleep-Disordered Breathing in Pulmonary Arterial Hypertension?

Clinical Trials Update

Pulmonary Hypertension and Chronic Obstructive Pulmonary Disease: When Is It Out of Proportion?

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis

Hypoxic Pulmonary Vasoconstriction and Chronic Lung Disease

Pulmonary Hypertension Roundtable: COPD, IPF, and Pulmonary Hypertension: A Clinician's Dilemma

PHPN: O₂ Trials and Tribulations

LOOKING BACK

News to Use

PHA Classroom

PHA Resources for Patients

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