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Purpose: Low pulmonary vascular resistance (PVR) is crucial to patients with a Fontan circulation. Increase of the PVR will result in a decrease of lung flow and cardiac output. Plasma endothelin-1 level, a vasoconstrictor which increases PVR, is elevated in patients with Fontan circulation. We aimed to determine whether bosentan, an endothelin-1 receptor antagonist, increases cardiac outputin Fontan patients by reducing the PVR.Methods: The study is a prospective, multicenter randomized open label trial. Patients were assigned to six months bosentan treatment or three months without treatment followed by six months of bosentan treatment. The current analysis evaluates results of the first three months. Aside of cardiac output on trans-thoracic echocardiography (TTE), were NYHA functional class, NT-pro-BNP level and SF-36 quality of life (QoL) scores endpoints.Results: We included 32 patients (mean age 32 ± 9 years, 59% male, 86% NYHA I-II). There was no significant difference in change of mean cardiac output, ejection fraction, NYHA functional class or QoL physical axis scores between treated and untreated patients (Table). In addition, qualitative ventricular function by ‘global eyeballing’ was unchanged. NT-pro-BNP levels were not significantly increased in the treatment group and were decreased in the untreated group. Whereas QoL mental axis scores improved at three months follow up for treated patients, no improvement was seen for untreated patients.Conclusion: This preliminary data shows subjective benefit of bosentan treatment in Fontan patients, which was not accompanied by objective improvement. However, the treatment effect may need time to emerge in this ongoing study.