• Matthias Held, Maria Grün, Regina Holl, Gudrun Hübner, Ralf Kaiser, Sabine Karl, Martin Kolb, Hans Joachim Schäfers, Heinrike Wilkens, and Berthold Jany.
• Department of Internal Medicine, Medical Mission Hospital, Academic Teaching Hospital, Julius Maximilian University of Würzburg, Würzburg, Germany.
• Respiration. 2014 Jan 1; 87 (5): 379-87.

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a serious complication of pulmonary embolism (PE). Taking into account the reported incidence of CTEPH after acute PE, the number of patients with undiagnosed CTEPH may be high.ObjectivesWe aimed to determine if cardiopulmonary exercise testing (CPET) could serve as complementary tool in the diagnosis of CTEPH and can detect CTEPH in patients with normal echocardiography.MethodsAt diagnosis, we analyzed the data of CPET parameters in 42 patients with proven CTEPH and 51 controls, and evaluated the performance of two scores.ResultsVE/VCO2 slope, EQO2, EQCO2, P(A-a)O2, end-tidal partial pressure of CO2 at anaerobic threshold (PETCO2) and capillary to end-tidal carbon dioxide gradient [P(c-ET)CO2] were significantly different between patients with CTEPH and controls (p < 0.001). P(c-ET)CO2 was the single parameter with the highest sensitivity (85.7%) and specificity (88.2%). A score combining VE/VCO2 slope, P(A-a)O2, P(c-ET)CO2, PETCO2 [4-parameter-CPET (4-P-CPET) score] reached a sensitivity of 83.3% and a specificity of 92.2% after cross-validation. In 42 patients with CTEPH, echocardiography identified PH in 29 patients (69%), but it was normal in 13 patients (31%). All patients with normal or unmeasurable right ventricular systolic pressure had a pathological CPET. Twelve of the 13 patients (92%) were detected by both CPET scores.ConclusionCPET is a useful noninvasive diagnostic tool for the detection of CTEPH in patients with suspected PH but normal echocardiography. The 4-P-CPET score provides a high sensitivity with the highest specificity.

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