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. 2021 Dec 10;2021(1):478-484. doi: 10.1182/hematology.2021000282

Table 1.

Comparison of diagnostic tests used in the evaluation for CTEPH

Diagnostic test Findings in CTEPH Advantages Disadvantages/limitations
Imaging
 Echocardiogram • Evidence of PH or RH strain: RV dilation, RV systolic dysfunction, RA dilation, PASP elevation, flattening of interventricular septum • Non-invasive
• No exposure to radiation or contrast dye
• Not specific or sensitive to CTEPH
• Misses CTED
 VQ • Mismatched defects in perfusion and ventilation defects
• Heterogeneity of perfusion
• Highest sensitivity to rule out CTEPH
• Less radiation exposure
• No contrast dye exposure
• Limited access as performed in nuclear medicine
• Unable to provide alternative diagnosis
• Sensitive but not specific for CTEPH
 CT PA • Pulmonary arteries: PA dilation, webs/bands, eccentric filling defects, mural thrombi, luminal narrowing with poststenotic dilation, complete occlusion, pouch defects
• Heart: RV dilation, septal flattening
• Lungs: mosaic attenuation, large bronchial artery collaterals
• Defining vascular anatomy can aid in surgical assessment
• Provides data on screening for concomitant lung disease/alternate diagnosis that can aid in surgical risk assessment
• CTEPH findings can sometimes be subtle and require expertise and attention to diagnose
• Exposure to IV contrast
• Exposure to radiation
• Less sensitive than VQ (neg CT does not exclude CTEPH)
Invasive testing
 Pulmonary angiogram • Ring lesions/ring-like stenosis
• Poststenotic dilation
• Total occlusion
• Vascular webs
• Provides complete hemodynamic assessment and aids in surgical planning • Invasive
 RH catheterization • mPAP ≥25 mm Hg
• Wedge ≤15 mm Hg
• Provides complete hemodynamic assessment and aids in surgical planning • Invasive
Functional testing
 Cardiopulmonary exercise testing • Increased dead space ventilation with widening A-a gradient, flattened stroke volume in response to exercise • Can detect limitations in function and cardiopulmonary response to exercise • Requires specialized testing unit
• Requires arterial blood gas measurement
 6-minute walk test • Functional limitations and decreased oxygen saturation with exercise • Simple to perform, low cost, minimal risk • Not specific to CTEPH

IV, intravenous; PASP, pulmonary artery systolic pressure; RA, right atrium.