Update On The Diagnosis And Treatment Of Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension(CTEPH)is characterized by mechanical obstruction of the proximal pulmonary arteries and remodeling of distal pulmonary artery.Progressive pulmonary vascular resistance and severe pulmonary hypertension are the main clinical characters of CTEPH,which eventually leads to right heart failure.It has a pool prognosis without treatment.Thought the pathogenesis of CTEPH is unclear,it has been found that the acquired thrombophilic,chronic inflammation,fibrin structure variation and vascular development defects may related to CTEPH.With the improvement of pulmonary artery endarterectomy(PEA),balloon pulmonary anguoplasty(BPA),pulmonary vasodilators,CTEPH is potentially curable.We provide an overview of current understanding of epidemiology,pathogenesis,diagnosis and treatment of CTEPH.