Drug Therapy in Patients with Chronic Thromboembolic Pulmonary Hypertension

Background: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from the obstruction of predominantly major pulmonary vessels by organized blood clots and is one of the causes of pulmonary hypertension. The disease is still underdiagnosed and the true prevalence is not clear. The objective of this study was to determine the clinical characteristics and current management of patients with CTEPH. Methods: This cross sectional study was conducted in 2011till 2017 on 70 patients with CTEPH at Masih Daneshvari Hospital. World Health Organization (WHO) functional class, six minute walking test (6MWT), pulmonary artery pressure (PAP) and plasma NT-ProBNP level were measured, before, during and after treatment. Results: Results showed that in Patients which were treated with prostanoids, Endothelin receptor antagonist (ERAs), phosphodiesterase type 5 inhibitors (PDE-5i) and soluble guanylate cyclase (sGC) stimulators, NYHA score, PAP, NT-ProBNP and the distance walked in 6MWD significantly improved. Conclusion: our results suggest that drug therapy in patients with CTEPH leads to a reduction in the functional class and pulmonary arterial pressure and prevents progression of the disease of the patients during the course of treatment.