Update on the roles of imaging in the management of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH), classified as group 4 pulmonary hypertension (PH), is caused by stenosis and obstruction of the pulmonary arteries by organized thrombi that are incompletely resolved after acute pulmonary embolism. The prognosis of patients with CTEPH is poor if untreated; however, in expert centers with multidisciplinary teams, a treatment strategy for CTEPH has been established, dramatically improving its prognosis. CTEPH is currently not a fatal disease and is the only curable form of PH. Despite these advances and the establishment of treatment approaches, early diagnosis is still challenging, especially for non-experts, for several reasons. One of the reasons for this is insufficient knowledge of the various diagnostic imaging modalities, which are essential in the clinical practice of CTEPH. Imaging modalities should detect the following pathological findings: lung perfusion defects, thromboembolic lesions in pulmonary arteries, and right ventricular remodeling and dysfunction. Perfusion lung scintigraphy and catheter angiography have long been considered gold standards for the detection of perfusion defects and assessment of vascular lesions, respectively. However, advances in imaging technology of computed tomography and magnetic resonance imaging have enabled the non-invasive detection of these abnormal findings in a single examination. Cardiac magnetic resonance (CMR) is the gold standard for evaluating the morphology and function of the right heart; however, state-of-the-art techniques in CMR allow the assessment of cardiac tissue characterization and hemodynamics in the pulmonary arteries. Comprehensive knowledge of the role of imaging in CTEPH enables appropriate use of imaging modalities and accurate image interpretation, resulting in early diagnosis, determination of treatment strategies, and appropriate evaluation of treatment efficacy. This review summarizes the current roles of imaging in the clinical practice for CTEPH, demonstrating the characteristic findings observed in each modality.