Improved low-risk criteria scores for combination therapy of sildenafil and generic bosentan in patients with congenital heart disease with severe pulmonary hypertension: A prospective open label study

摘要

Objective We evaluated the efficacy and safety of the bosentan as a sequential add-on therapy with sildenafil in pulmonary arterial hypertension with congenital heart disease (PAH-CHD) patients. Material and method Twenty patients who were receiving sildenafil were given generic bosentan for up to a year. Hemodynamic data was collected from cardiac catheterization at pretreatment and at three months. Comparisons were made between the total scores of the four, low-risk criteria adapted from the 2015 ESC/ERS pulmonary hypertension guidelines, which are: 1) WHO functional class of I or II, 2) 6MWD of more than 440?m, 3) right atrial pressure of less than 8?mm Hg, and 4) cardiac index ≥2.5?L/min/m ~(2), performed at the beginning of therapy, 3-months, 6-months, and 1?year. Results Patients’ average age was 27?±?11?years old (12–53). PVRi decreased from 16.7?±?9.5 to 12.7?±?10.3 Wood unit (WU) m2 (p?=?0.025) and PVRi/SVRi decreased from 0.69?±?0.33 to 0.49?±?0.32 (p?=?0.001). During the follow-up, the composite scoring of the low risk scores for 19 patients was increased significantly from 1.8?±?1.0 at baseline to 2.3?±?0.9 at 3?months, to 2.9?±?0.8 at 6?months, and 3?±?0.7 at 1?year (p?=?0.001). Conclusion We demonstrated intermediate term benefits for generic bosentan as an add-on therapy to sildenafil in patients with PAH-CHD by improving PVRi, and PVRi/SVRi at three months. A significant improvement was also seen in the combined scores of the low-risk criteria from below 2 to 3 at one year (p?=?0.001). Thai Clinical Trials Registry (TCTR): TCTR identification number is TCTR20200506006.