Case report: the possibility of specific therapy optimization by switching from Bosentan to Macitentan in a patient with congenital heart disease and Eisenmenger syndrome

A patient diagnosed with pulmonary arterial hypertension (PAH) associated with congenital heart disease (ventricular septal defect), Eisenmenger syndrome was started with PAH-specific therapy with endothelin receptor antagonist (ARE) - bosentan. With the treatment, the patient's condition remained stable, but by the second year of therapy, negative dynamics was observed in the form of increased shortness of breath and decreased exercise tolerance and an increase in the right heart. Therapy with bosentan is replaced by macitentan. One year after the change of therapy, an improvement in the functional status was noted. In addition, the patient was successfully performed gynecological surgery with general anesthesia during matsitentan therapy.