Progress on the pathogenesis of chronic thromboembolic pulmonary hypertension

Abstract

Chronic thrombotic pulmonary hypertension (CTEPH) is a rare progressive pulmonary vascular disease, which is classified as World Health Organization (WHO) functional class Ⅳ pulmonary arterial hypertension (PAH). The diagnosis was based on mean pulmonary arterial pressure ≥25 mmHg(1 mmHg=0.133 kPa) and pulmonary arterial wedge pressure ≤15 mmHg measured by right heart catheterization, with the under-utilization of radionuclide ventilation/perfusion scan and regular anticoagulation therapy for at least three months. The incidence of CTEPH is low, at 4 cases per million population per year. Despite the in-depth research on CTEPH in recent years, the strengthening of clinical diagnostic awareness, and the emergence of treatment methods such as pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA), the prognosis of CTEPH patients has been dramatically improved. However, the pathogenesis of CTEPH is unclear, and the lack of effective early diagnostic methods has become a bottleneck limiting the improvement of clinical diagnosis and treatment. In this paper, we reviewed the current research progress on the pathogenesis of CTEPH to increase the understanding of CTPTH disease.