Abstract Introduction Plastic bronchitis is a rare and serious complication of Fontan procedure for double outlet right ventricle (DORV). It occurs in 4-5% of individuals and is associated with a significant morbidity and mortality. Case Presentation A 5-year-old male with DORV, ventricular septal defect, pulmonary stenosis who underwent atrial septostomy, right sided glen procedure (complicated by persistent chylous pleural effusions), Fontan procedure with pulmonary artery plasty. Approximately, two years following the procedure he developed acute hypoxic respiratory failure due to airway casts and was diagnosed with plastic bronchitis requiring multiple bronchoscopies, airway clearance therapy and a low-fat diet. He was subsequently readmitted after a cardiac arrest attributed to acute hypoxemia secondary to tracheal obstruction. He underwent laryngoscopy with attempts to remove casts using rigid suction, optical forceps and laryngeal microdebriders with incomplete success. A rigid bronchoscopy was performed and cryoprobe used to debulk and remove numerous lymphatic casts occluding the trachea, right and left main stem with resultant patency of airways except left lower lobe. Unfortunately, despite VA-ECMO support for cardiogenic shock his condition deteriorated and he passed away. Discussion The Fontan procedure reroutes systemic venous return directly to the pulmonary arteries, bypassing the right ventricle. Plastic bronchitis typically develops 2-3 years post-operatively. Risk factors include a dominant right single ventricle, older age at Fontan completion, and post-operative pleural effusions—all contributing to increased lymphatic dysfunction.Chronically elevated central venous pressures alter Starling forces, disrupting normal lymphatic drainage from the thoracic duct. Resultant lymphatic congestion and insufficiency can create aberrant lymphatic-bronchial connections, leading to lymphatic leakage into the airways. This leakage triggers airway inflammation and formation of proteinaceous casts, manifesting as productive cough, hypoxemia, or acute respiratory failure.Management targets both symptom relief and correction of hemodynamic and lymphatic abnormalities. These include bronchoscopy for cast removal, bronchodilators, mucolytics, and inhaled tissue plasminogen activator (tPA) for cast dissolution. Adjunctive measures include pulmonary vasodilators and diuretics to lower central venous pressure, low-fat diet to reduce lymphatic flow, and catheter-based lymphatic embolization or thoracic duct decompression. Despite these interventions, prognosis remains guarded with 5-year mortality rate approximately 15-30%, with higher risk in those with early onset after surgery. Definitive therapy is a heart transplant. References: Ghosh RM et al. J Am Heart Assoc. 2020;9(7):e015318.Sharma VJ et al. J Thorac Cardiovasc Surg. 2021;161(6):2158-2165.e4.Harteveld LM et al. Interact Cardiovasc Thorac Surg. 2020;30(6):846-853.Mackie AS et al. Can J Cardiol. 2022;38(7):988-1001.Roché-Rodríguez M et al. J Cardiothorac Vasc Anesth. 2022;S1053-0770(21)00631-5. This abstract is funded by: none
Haque et al. (Fri,) studied this question.