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

Abstract

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.