“…Prevention of cast formation is the main objective. Treatment has been established by previous case reports and includes bronchial cleaning with bronchoscopy, management of increased venous pressure and aggressive pulmonary vasodilation with sildenafil and bosentan [1], as well as inhaled and systemic steroids due to their muco-regulatory properties, especially in PB type I [11], use of mucolytics (dornase alpha, N-acetylcysteine, hypertonic saline solution) [1,12], intravenous or inhaled heparin similar to its use in cases of injury by smoke inhalation, (intravenously improves oxygenation, decreases barotrauma and airway edema; the mechanism of action is unclear [13]), followed by fibrinolytics such as urokinase and inhaled tissue plasminogen activator [14]. In patients with poor response to treatment, it is necessary to remove the Fontan or perform a heart transplant [1,9] and in adults, lymphatic percutaneous embolization is described to attenuate the loss of lymph, with partial or complete resolution of the symptoms [15].…”