Plastic Bronchitis: A Rare Complication After Fontan Surgery

Plastic bronchitis (hard, sticky mucous plugs) is a medical emergency where obstructing bronchial casts may cause asphyxia. 9-year-old boy presented with choking cough fits, producing rubbery sputum 1 year after Fontan operation. He had right atrial isomerism, unbalanced complete atrioventricular septal defect, pulmonary atresia, and received bosentan therapy before a fenestrated Fontan operation. Catheterization at presentation revealed oxygen saturation 84%, mean pulmonary artery (PA) pressure 18-19 mmHg, and pulmonary vascular resistance 3.5 WU. Narrow PA segment between the bilateral cavo-pulmonary anastamoses was stented. Medical treatment consisted of intravenous fluconazole, ceftriaxone; oral clarithromycin, prednisolone, sirolimus; inhaler salbutamol, budezonide, pulmozyme, heparin; and middle chain triglyceride diet. There were no casts in the bronchoscopy. One year after his discharge, he is stable, with no recurrences. Elevated PA pressure and failing Fontan circulation needs to be evaluated if plastic bronchitis is seen after Fontan surgery. Urgent medical treatment and bronchoscopy for bronchial casts is very crucial in these patients. Sirolimus may be a new option in medical treatment.