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Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: An evidence-based review

https://doi.org/10.1016/j.healun.2009.09.003Get rights and content

Background

There is an increasing recognition of the potential value of cardiopulmonary exercise testing (CPX) in patients with pulmonary hypertension (PH). Key CPX characteristics in these patients include: (1) a diminished aerobic capacity; (2) an abnormally elevated minute ventilation–carbon dioxide production relationship; and (3) an abnormally diminished partial pressure of end-tidal carbon dioxide. Given the burgeoning number of original research investigations utilizing CPX in patients with PH, a summation of the presently available body of literature seems timely.

Methods

A literature search was conducted in pubmed using “cardiopulmonary exercise testing” and “pulmonary arterial hypertension” as key phrases. Only studies conducting exercise testing with simultaneous ventilatory expired gas analysis in subjects with a confirmed diagnosis of pulmonary arterial hypertension were included. Twenty-three investigations were included in this review. Nineteen of the investigations assessed cohorts with resting pulmonary arterial hypertension as the sole diagnosis. Two investigations assessed subjects with chronic obstructive pulmonary disease and pulmonary arterial hypertension: one assessed subjects with pulmonary fibrosis and pulmonary arterial hypertension, and another included groups with exercise-induced pulmonary arterial hypertension and resting pulmonary arterial hypertension.

Results

Collectively, these investigations indicate variables obtained from CPX: (1) reflect varying degrees of PH severity; (2) positively respond to several pharmacologic and surgical interventions; and (3) may provide prognostic value.

Conclusions

Currently, CPX is not widely utilized in patients with PH. Although more research is required in a number of areas, the present evidence-based review indicates this exercise testing technique may provide valuable information in the PH population.

Section snippets

Link Between PH Pathophysiology and Abnormal CPX Response

The ventilatory expired gas abnormalities precipitated by PH are multifactorial and associated with disease severity.43, 44 Increased pulmonary artery pressure (PAP), the primary pathophysiologic consequence of this condition, creates a ventilation–perfusion mismatch (i.e., acceptable ventilation/diminished perfusion). This results in an increase in physiologic dead space, which, from a ventilatory expired gas perspective, is reflected by an elevated Ve/Vco2 ratio or slope and diminished

Evidence Supporting Utility of CPX in Patients with PH

A literature search was conducted in pubmed using “cardiopulmonary exercise testing” and “pulmonary arterial hypertension” as key phrases. Only studies conducting exercise testing with simultaneous ventilatory expired gas analysis in subjects with a confirmed diagnosis of pulmonary arterial hypertension were included. The 23 presently available investigations addressing this area of research are listed in Table 1. Nineteen of the investigations assessed cohorts with resting pulmonary arterial

Safety of CPX in patients with PH: sub-maximal vs maximal exercise testing

None of the investigations listed in Table 1 reported adverse events with CPX. Similarly, no adverse events with CPX were reported in a small pediatric cohort with PH.72 Severe PH with accompanying syncopal episodes, cardiac arrhythmias or acute right ventricular failure do, however, serve as contraindications to maximal exercise testing.41 Both the Ve/Vco2 ratio and PETco2 demonstrate abnormalities at rest and sub-maximal exercise that are reflective of PH disease severity and potentially

Disclosure Statement

The authors have no conflicts of interest to disclose.

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