Abstract

The right ventricle (RV) is more sensitive to an increase in afterload than the left ventricle (LV), and RV afterload during exercise increases more easily than LV afterload. Pulmonary arterial hypertension (PAH)-specific therapy has improved pulmonary hemodynamics at rest; however, the pulmonary hemodynamic response to exercise is still abnormal in most patients with PAH. In these patients, RV afterload during exercise could be higher, resulting in a greater increase in RV wall stress. Recently, an increasing number of studies have indicated the short-term efficacy of exercise training. However, considering the potential risk of promoting myocardial maladaptive remodeling, even low-intensity repetitive exercise training could lead to long-term clinical deterioration. Further studies investigating the long-term effects on the RV and pulmonary vasculature are warranted. Although the indications for exercise training for patients with PAH have been expanding, exercise training may be associated with various risks. Training programs along with risk stratification based on the pulmonary hemodynamic response to exercise may enhance the safety of patients with PAH.

Highlights

  • Pulmonary arterial hypertension (PAH), defined as resting mean pulmonary arterial pressure > 20 mmHg, pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 wood units in the absence of other causes of pre-capillary pulmonary hypertension (PH), is a cardiopulmonary disorder characterized by a progressive increase in PVR, resulting in right ventricular (RV) failure [1,2]

  • We found that exercise capacity was reduced and pulmonary hemodynamic response during incremental submaximal exercise testing was abnormal in most patients with PAH, even after hemodynamics at rest were improved with PAH-specific therapy [9]

  • Even after hemodynamics at rest are improved with PAH-specific therapy, RV afterload during exercise could be high due to residual impairment of the pulmonary hemodynamic response in patients with PAH

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Summary

Introduction

Pulmonary arterial hypertension (PAH), defined as resting mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 wood units in the absence of other causes of pre-capillary pulmonary hypertension (PH), is a cardiopulmonary disorder characterized by a progressive increase in PVR, resulting in right ventricular (RV) failure [1,2]. PAH-specific therapy has improved the pulmonary hemodynamics at rest, exercise capacity, quality of life, and prognosis of patients in the last two decades [5,6,7]. We found that exercise capacity was reduced and pulmonary hemodynamic response during incremental submaximal exercise testing was abnormal in most patients with PAH, even after hemodynamics at rest were improved with PAH-specific therapy [9]. In these patients, RV afterload during exercise could be higher, and exercise has the potential to promote myocardial remodeling. This review focuses on training programs to enhance the safety of patients with PAH

Acute and Chronic RV Responses to Exercise
Pathophysiology of Exercise Intolerance in Patients with PAH
Conclusions
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