CHEST Guidelines-Evaluation-and-Management-of-Chronic-Thromboemboli

Evaluation-and-Management-of-Chronic-Thromboemboli

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension resulting from unresolved obstructions in pulmonary arteries due to persistent thromboembolic disease. Despite its potential underrecognition, particularly in patients without a history of venous thromboembolism (VTE), CTEPH is potentially curable via pulmonary thromboendarterectomy (PTE) surgery, which has a low mortality rate in expert centers. However, over one-third of patients with CTEPH may be deemed inoperable due to distal disease or other factors, for whom effective treatments include balloon pulmonary angioplasty (BPA) and pharmacotherapy, notably with riociguat.<br /><br />CTEPH is differentiated from other forms of pulmonary hypertension through diagnostic imaging such as V/Q scintigraphy and confirmed via pulmonary angiography and right heart catheterization. Key risk factors include a history of unprovoked pulmonary embolism, antiphospholipid antibodies, and certain medical conditions like malignancy and hypothyroidism. The disease is classified based on the level of vascular obstruction severity, guiding treatment decisions. <br /><br />Recent advancements have improved surgical techniques for more distal PTE and increased the scope of BPA. BPA, while initially risky, now presents favorable outcomes in selected inoperable patients. Long-term pharmacotherapy, primarily with anticoagulants and increasingly direct oral anticoagulants (DOACs), is essential. <br /><br />Multidisciplinary care is integral to optimize treatment outcomes, encompassing a combination of surgery, BPA, and medical therapy tailored to individual patient profiles. Ongoing research continues to explore new therapeutic strategies, particularly for CTEPD without resting pulmonary hypertension, as its natural history and optimal treatment remain undetermined. Despite current therapeutic innovations, there remain gaps in understanding the full landscape of CTEPH, necessitating continued research and specialized care.

Keywords

CTEPH

pulmonary hypertension

thromboembolic disease

pulmonary thromboendarterectomy

balloon pulmonary angioplasty

riociguat

diagnostic imaging

pharmacotherapy

multidisciplinary care

direct oral anticoagulants