EXPRESS: Optimizing CTEPH diagnosis and assessment using advancing imaging modalities

Imaging is key to nearly all aspects of chronic thromboembolic pulmonary hypertension including management for screening, assessing eligibility for pulmonary endarterectomy, and post-operative follow-up. While ventilation/perfusion scintigraphy, the gold standard technique for chronic thromboembolic pulmonary hypertension screening, can have excellent sensitivity, it can be confounded by other etiologies of pulmonary malperfusion, and does not provide structural information to guide operability assessment. Conventional computed tomography pulmonary angiography has high specificity, though findings of chronic thromboembolic pulmonary hypertension can be visually subtle and unrecognized. In addition, computed tomography pulmonary angiography can provide morphologic information to aid in pre-operative workup and assessment of other structural abnormalities. Advances in computed tomography imaging techniques, including dual-energy computed tomography and spectral-detector computed tomography, allow for improved sensitivity and specificity in detecting chronic thromboembolic pulmonary hypertension, comparable to that of ventilation/perfusion scans. Furthermore, these advanced computed tomography techniques, compared with conventional computed tomography, provide additional physiologic data from perfused blood volume maps and improved resolution to better visualize distal chronic thromboembolic pulmonary hypertension, an important consideration for balloon pulmonary angioplasty for inoperable patients. Electrocardiogram-synchronized techniques in electrocardiogram-gated computed tomography can also show further information regarding right ventricular function and structure. While the standard of care in the workup of chronic thromboembolic pulmonary hypertension includes a ventilation/perfusion scan, computed tomography pulmonary angiography, direct catheter angiography, echocardiogram, and coronary angiogram, in the future an electrocardiogram-gated dual-energy computed tomography angiography scan may enable a "one-stop" imaging study to guide diagnosis, operability assessment, and treatment decisions with less radiation exposure and cost than traditional chronic thromboembolic pulmonary hypertension imaging modalities.