State of artCardiopulmonary exercise testing in patients with pulmonary arterial hypertension: An evidence-based review
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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