Overview of Epidemiology, Pathophysiology, Risk factors, Diagnosis and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the curable forms of pulmonary hypertension, but CTEPH can be lethal without treatment. The detection of risk factors may lead to earlier diagnosis and potentially curative interventions. Although CTEPH can be considered as a disorder of misguided vascular remodeling after pulmonary embolism (PE), as many as 25% to 30% of patients with CTEPH are diagnosed without a prior clinical history of PE. Pulmonary endarterectomy (PEA) is the gold standard treatment for CTEPH, but not all patients with CTEPH are operable. Recently, in addition to medical therapy, there is emerging treatment option called balloon pulmonary angioplasty (BPA) for those inoperable patients.