Volume 23: Issue 1

Exercise intolerance is a common feature of many cardiopulmonary diseases including pulmonary hypertension (PH) and sleep disordered breathing (SDB), which includes obstructive sleep apnea and obesity hypoventilation syndrome. Physiologic abnormalities in both PH and SDB can drive exercise intolerance, and biological mechanisms overlap among the conditions including systemic inflammation, oxidative stress, metabolic dysfunction, and endothelial dysfunction. Despite this understanding, evidence establishing clear causal relationships among PH, SDB, and exercise intolerance is lacking. Data show that treatment of SDB may improve exercise capacity, and exercise training likely improves SDB, although these relationships specifically in PH remain understudied. In this manuscript, we summarize existing data of mechanisms and clinical observations in PH, SDB and exercise and identify gaps and opportunities for future investigation.

Pulmonary hypertension is characterized by significant remodeling of the pulmonary vasculature, leading to raised pulmonary vascular resistance. Despite advances in medical therapy, decreased exercise tolerance remains a predominant symptom experienced by patients. Reduced exercise capacity has been shown to have implications on quality of life and prognosis. There is growing acknowledgment that the etiology of exercise tolerance is multifactorial with cardiac, respiratory, and skeletal muscle contributors. There has been a shift in management approach with exercise training now included as a Class 1 recommendation indication in recent guidelines. In this review, we summarize the literature on the pathophysiology of exercise intolerance in pulmonary hypertension and then describe the literature assessing the safety and efficacy of inspiratory muscle and exercise training in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.

Reduced exercise tolerance stands as the foremost symptom, profoundly impacting the lives of those grappling with pulmonary arterial hypertension (PAH). This decline stems from both pulmonary and cardiac irregularities. Nonetheless, there is a burgeoning recognition that dysfunction within peripheral skeletal muscles (SKMs) significantly contributes to compromised exercise capacity. Consequently, the morphological and functional impairments of SKMs, coupled with microvascular loss, proinflammatory states, and oxidative disorders, play substantial roles in limiting exercise capacity in PAH. Regrettably, these facets have only undergone partial scrutiny. Thus, this review aims to spotlight the current body of literature concerning SKM dysfunctions in PAH and pinpoint knowledge gaps warranting further exploration to deepen our comprehension of SKM dysfunction and exercise intolerance in PAH.

Catherine Avitabile and Aimee Layton asked questions of their colleagues from around the world on their experiences with exercise recommendations in PH patients of varying ages. They discussed patients’ and their families’ views on physical activity, benefits and risks of engaging in exercise, and resources for both health care providers and patients.

Avitabile/Layton: Do you find most of your patients exercise regularly or are physically active when you talk to them about their daily routines?

Chia: Most are quite sedentary.

Benjamin/Grünig: Patients with pulmonary hypertension are often very active or used to be very active before the

Full Issue PDF

Editor’s Memo

Guest Editors’ Memo

Sleep Disordered Breathing and Exercise in Pulmonary Hypertension

Pulmonary Hypertension: Exercise Intolerance and the Benefits of Respiratory Muscle and Exercise Training

Skeletal Muscle Structural and Functional Impairments as Important Peripheral Exercise Intolerance Determinants in Pulmonary Arterial Hypertension

PH Roundtable: Recommending Exercise in Pulmonary Hypertension: Adult and Pediatric Perspectives

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