The role of cardiopulmonary exercise testing and training in patients with pulmonary hypertension: making the case for this assessment and intervention to be considered a standard of care

Introduction: Pulmonary hypertension (PH) is a broad pathophysiological disorder primarily characterized by increased pulmonary vascular resistance due to multiple possible etiologies. Patients typically present with multiple complaints that worsen as disease severity increases. Although initially discouraged due to safety concerns, exercise interventions for patients with PH have gained wide interest and multiple investigations have established the effective role of exercise training in improving the clinical profile, exercise tolerance, and overall quality of life. Areas covered: In this review, we discuss the pathophysiology of PH during rest and exercise, the role of cardiopulmonary exercise testing (CPX) in the diagnosis and prognosis of PAH, the role of exercise interventions in this patient population, and the expected physiological adaptations to exercise training. Expert opinion: Exercise testing, in particular CPX, provides a wealth of clinically valuable information in the PH population. Moreover, the available evidence strongly supports the safety and efficacy of exercise training as a clinical tool in improving exercise tolerance and quality of life. Although clinical trials investigating the role of exercise in this PH population are relatively few compared to other chronic conditions, current available evidence supports the clinical implementation of exercise training as a safe and effective treatment modality.