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

ABSTRACT

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.

KEYWORDS:

• Exercise
• pulmonary hypertension
• cardiopulmonary exercise testing

Article highlights

• Patients with PH have a mean time of 3 years from initial symptom onset to diagnosis resulting in significant treatment delays and stressing the importance of early detection and intervention.

• Multiple pathophysiological mechanisms contribute to exercise intolerance in PH with increased PVR being a central complication. This increase in PVR eventually leads to reduced pulmonary perfusion and V/Q mismatch which contribute to ventilatory inefficiency and exercise intolerance.

• CPX can provide a wealth of information to assist with the diagnosis and prognosis of PH especially in early stages of disease when symptoms are masked during rest.

• Exercise training for patients with PH is generally safe, been shown to improve exercise capacity, quality of life, and has been recommended as an add-on therapy for clinically stable patients by multiple professional organizations.

• Further research is required to identify the optimum exercise prescription for patients with PH as well as explore the mechanistic adaptations to exercise observed in this patient population.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was funded in part by NIH training grant T32-HL139439 (AS).