Patients with CTED showed an abnormal pulmonary vascular response to exercise and a decreased ventilatory efficiency. Responses after PEA point to restoration of right ventricle stroke volume response and ventilatory efficiency.

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V'E /V'CO2 , the only CPET parameter associated with elevated sPAP, appears a potentially useful non-invasive marker for early detection of pulmonary vascular impairment, and therefore may be of use for a more accurate prognostic assessment in IPF patients.

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Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as precapillary pulmonary hypertension (PH) as assessed by right heart catheterization, and results from incomplete resolution of the vascular obstruction associated with acute pulmonary embolism (PE). Pulmonary thromboendarterectomy (PTE) is the therapy of choice for CTEPH patients with surgically accessible thrombi. Although associated with potential risks, PTE has been found to improve, and in many cases normalize pulmonary hemodynamics, functional status, and long-term survival. It is critical to undergo careful diagnosis and preoperative selection of patients who will most likely benefit from surgery. We have used published literature along with our personal experiences to review diagnosis of CTEPH and evaluation in advance of the PTE procedure. In patients with PH or suspected PH, a complete diagnostic workup should be performed to identify the underlying etiology of the disease. Pulmonary angiography and right heart catheterization are the preferred assessment tools to diagnose CTEPH. PTE remains the treatment of choice, and for further evaluation of operability and preoperative risk patients should be referred to a CTEPH expert center.