Multidisciplinary Approach to Chronic Thrombo embolic Pulmonary Hypertension: Role of Radiologists

Management of chronic thromboembolic pulmonary hy-pertension (CTEPH) should be determined by a multidisci-plinary team, ideally at a specialized CTEPH referral center. Radiologists contribute to this multidisciplinary process by helping to confirm the diagnosis of CTEPH and delineating the extent of disease, both of which help determine a treat-ment decision. Preoperative assessment of CTEPH usually employs multiple imaging modalities, including ventila-tion-perfusion (V/Q) scanning, echocardiography, CT pul-monary angiography (CTPA), and right heart catheteriza-tion with pulmonary angiography. Accurate diagnosis or ex-clusion of CTEPH at imaging is imperative, as this remains the only form of pulmonary hypertension that is curative with surgery. Unfortunately, CTEPH is often misdiagnosed at CTPA, which can be due to technical factors, patient -re-lated factors, radiologist-related factors, as well as a host of disease mimics including acute pulmonary embolism, in situ thrombus, vasculitis, pulmonary artery sarcoma, and fi-brosing mediastinitis. Although V/Q scanning is thought to be substantially more sensitive for CTEPH compared with CTPA, this is likely due to lack of recognition of CTEPH findings rather than a modality limitation. Preoperative evaluation for pulmonary thromboendarterectomy (PTE) includes assessment of technical operability and surgical risk stratification. While the definitive therapy for CTEPH is PTE, other minimally invasive or noninvasive therapies also lead to clinical improvements including greater survival. Complications of PTE that can be identified at postopera-tive imaging include infection, reperfusion edema or injury, pulmonary hemorrhage, pericardial effusion or hemoperi-cardium, and rethrombosis. (C) RSNA, 2022