This review summarizes the diagnostic pathway and therapeutic options for chronic thromboembolic pulmonary hypertension, including surgical pulmonary endarterectomy, medical therapy, and balloon pulmonary angioplasty.
Supports multimodal CTEPH care pathways; leaves open comparative effectiveness of PEA, medical therapy, and BPA.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension resulting from obstruction of the pulmonary artery by fibrotic thromboembolic material, usually initiated by recurrent or incomplete resolution of pulmonary embolism. This distinct form of pulmonary hypertension is classified under Group 4 of the World Health Organization classification. Further investigations are usually initiated, with transthoracic echocardiography followed by right heart catheterisation and pulmonary angiography as the gold standard. Definitive treatment is usually in the form of surgical pulmonary endarterectomy. Inoperable CTEPH is medically treated with pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators and prostacyclin. Recent developments have made balloon pulmonary angioplasty a viable option as well.
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Chong et al. (2021) studied this question.
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