The surgical treatment for patients with endomyocardial fibrosis consists of resection of endocardial fibrotic tissue and replacement or repair of atrioventricular valve. Even after endocardiectomy and valvular remodeling, some patients exhibit very poor hemodynamic profile because of myocardial failure due to long-standing restriction of ventricle or ventricles. Here, we report a case of endomyocardial fibrosis in right ventricle who underwent endocardiectomy and valvular replacement followed by bi-directional cavopulmonary shunt to compensate weaning failure from cardiopulmonary bypass. The long-term outcome and the indication of bi-directional cavopulmonary shunt has not been confirmed, although it was effective for saving the life of patients with low cardiac output and acute right ventricular failure.