Volume 18: Issue 2

Pulmonary arterial hypertension (PAH) is a chronic disease that is associated with a significant and progressive limitation of exercise tolerance. The pathophysiological mechanisms of exercise intolerance during exercise are complex, multifactorial, and in fact not limited to the pulmonary circulation and the right ventricle. Disturbance of autonomic nervous function leads to an enhanced chemosensitivity, as well as respiratory and peripheral muscle weakness, and systemic endothelial dysfunction, which together play important roles in PAH pathophysiology and symptomatology. This article is focused on the different pathophysiological mechanisms of exercise intolerance in PAH, their interactions, and their relevance for clinical practice.

Background: Most pulmonary vascular disease (PVD) is poorly modifiable and incurable even with effective therapy. Therefore, adaptation to stress, the reserve of the cardiopulmonary system, is important for assessment of patient function and prognosis. Methods that assess the adaptation to stress, especially exercise, provide valuable insight into diagnosis, prognosis, and response to therapy.

Implications for Clinicians: We provide a comprehensive review of the indications, methodology, and interpretation, as well as practical information of the forms of provocative testing in PVD. We include 6-minute walk testing, noninvasive cardiopulmonary exercise testing (CPET), invasive CPET, and additional forms, including volume loading.

Conclusions: Through a clear understanding of the methodology in the assessment of PVD, the clinician can determine which of these “tools of the trade” are best suited to the individual patient and situation.

Exercise training as treatment has become well established in many cardiorespiratory conditions. This is also increasingly the case in pulmonary arterial hypertension, where several studies have demonstrated improvements in symptoms, exercise capacity and quality of life. There remains, however, much potential for development. Current research is focused on clarifying the mechanism of benefit in pulmonary hypertension and exploring strategies for both optimizing the treatment effect and widening access to this intervention.

WHAT IS PULMONARY REHABILITATION?

Pulmonary rehabilitation (PR) is a specialized physical therapy program to improve the quality of life for patients who have symptoms from chronic lung disease. The American Thoracic Society offers a formal definition of PR as a:

. . . comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies that include, but are not limited to, exercise training, education, and behavior change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviors.1

Although PR was originally developed to address the

Full Issue PDF

Memos

Editor's Memo

Exercise Pathophysiology in Pulmonary Arterial Hypertension—The Physiologic Explanation for Why Pulmonary Arterial Hypertension Does What It Does

Tools of the Trade: How Do You Perform and Interpret an Exercise Test?

Treating Pulmonary Arterial Hypertension With Exercise: The Role of Rehabilitative Medicine

Pulmonary Hypertension Roundtable: The Role of Exercise in Clinical Practice and Clinical Trials

Ask the Expert: What Is the Exercise “Prescription” That You Provide to Your Patients?

PH Grand Rounds: A Case of Pulmonary Hypertension Associated With High Cardiac Output State from Arteriovenous Fistula–Or Is It?

PRESENTATION

PH Professional Network: “Just Do It”: Practical Aspects of Pulmonary Rehabilitation Programs

WHAT IS PULMONARY REHABILITATION?

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