![]() 24 To compare pulmonary arterial pressure between patients with pulmonary arterial hypertension associated with congenital heart disease and patients with chronic thromboembolic PH, we used systolic pulmonary arterial pressure estimated by echocardiography. 23 The diagnosis chronic thromboembolic PH was established with pulmonary angiography and right heart catheterization (mean pulmonary arterial pressure >25 mmHg). In patients with congenital heart disease, pulmonary arterial hypertension was diagnosed by echocardiography (systolic pulmonary arterial pressure >40 mmHg). A 12-lead surface ECG was recorded of all patients. ![]() Patients were examined at baseline prior to treatment and re-examined after 1 year follow-up. Excluded were patients with Down syndrome, obstruction of the RV outflow tract, pulmonary valve or pulmonary arteries, or patients who were under treatment with prostacyclin, glibenclamide, or cyclosporin. In this retrospective open-label study, we analysed adult patients with pulmonary arterial hypertension associated with congenital heart disease ( n = 9) and patients with chronic thromboembolic PH ( n = 9). 12, 16, 19, 22 Therefore, the present study was designed to test the following hypotheses: (i) RV contraction duration at baseline is a predictor of improvement in exercise capacity and (ii) RV contraction duration shortening during treatment is associated with an increase in exercise capacity. 21 However, not all patients with PH show improvement of 6-MWD during treatment, and predictors for efficacy of bosentan treatment have not yet been identified. As an intermediate endpoint, the distance walked may translate to the ability to perform activities of daily living, exercise capacity, and quality of life in PH patients. 10, 12–20 The 6-MWD is frequently used in clinical practice to assess response to therapy in PH. 11 Consequently, bosentan treatment improves haemodynamics, 6 min walk distance (6-MWD), and survival in patients with various forms of PH. 10 Moreover, bosentan treatment resulted in a reduction of contraction duration leading to a reduction of leftward ventricular septal bowing and an improvement of LV early diastolic filling. ![]() Recently, it has been demonstrated that treatment with an endothelin receptor antagonist (bosentan) results in reverse remodelling of the pulmonary vascular wall leading to a decrease in pulmonary vascular resistance. 4–6 Prolongation of RV contraction duration is associated with elevated endothelin-1 levels. 1, 2 In addition, it has been demonstrated that in patients with PH, the duration of right ventricular (RV) contraction 3 is prolonged resulting in leftward ventricular septal bowing and impaired left ventricular (LV) early diastolic filling. Pulmonary hypertension (PH) is a progressive disease characterized by elevated endothelin-1 levels associated with vasoconstriction and structural changes in the pulmonary vascular bed. Pulmonary hypertension, Congenital heart disease, Chronic thromboembolic pulmonary hypertension, Echocardiography, Bosentan treatment Introduction
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