Fontan-Associated Plastic Bronchitis

Abstract

We read with interest the recent review highlighting the role of lymphatic dysfunction as a causative factor for the development of edema, pleural effusions, plastic bronchitis, and protein-losing enteropathy in patients with Fontan physiology.1RochéRodríguez M DiNardo JA. The lymphatic system in the Fontan patient-pathophysiology, imaging, and interventions: What the anesthesiologist should know.J Cardiothorac Vasc Anesth. 2022; 36: 2669-2678Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar Plastic bronchitis is characterized by the formation of large, rubbery plugs or “casts” in the tracheobronchial tree.2Schumacher KR Singh TP Kuebler J et al.Risk factors and outcome of Fontan-associated plastic bronchitis: A case-control study.J Am Heart Assoc. 2014; 3e000865Crossref PubMed Scopus (50) Google Scholar In Fontan surgery survivors, the incidence of plastic bronchitis is approximately 4%.3Caruthers RL Kempa M Loo A et al.Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis.Pediatr Cardiol. 2013; 34: 256-261Crossref PubMed Scopus (75) Google Scholar We recently encountered a unique case of plastic bronchitis. A 4-year-old boy underwent an extracardiac unfenestrated Fontan procedure using a polytetrafluoroethylene conduit. After the procedure, the transpulmonary and Fontan circuit pressures were 8-to-10 mmHg and 15-to-16 mmHg, respectively. After surgery, the patient was taken to the intensive care unit, but copious viscous tracheal secretions that required frequent suctioning and elevated airway pressures were observed. There was a simultaneous increase in chest tube drainage that appeared to be lymph fluid. The child was taken to the operating room for reexploration. During surgery, a further increase in airway pressure (>40 cmH2O) was observed, and it became difficult to ventilate the child. A rigid bronchoscopy was performed to evaluate the cause. The examination revealed erythematous mucosa, with a ball-like thick secretion near the carina that almost obstructed the tracheal lumen (Video 1). Biopsy forceps were used to extract the secretions, which were rubbery and consistent with plastic bronchitis (Fig 1). The secretions continued despite this intervention until a fenestration in the conduit was created. The child subsequently made an uneventful recovery. The risk factors for developing plastic bronchitis include prolonged chest tube drainage after surgery, chylothorax, and the development of aortopulmonary collaterals.2Schumacher KR Singh TP Kuebler J et al.Risk factors and outcome of Fontan-associated plastic bronchitis: A case-control study.J Am Heart Assoc. 2014; 3e000865Crossref PubMed Scopus (50) Google Scholar Plastic bronchitis after Fontan surgery is an adverse effect of suboptimal adaptation to cavopulmonary circulation that requires immediate correction because the thick cast can obstruct the airway completely, as observed in this patient. Rigid bronchoscopy may be needed to identify and facilitate the removal of rubbery secretions and prevent asphyxiation.4Ishman S Book DT Conley SF et al.Plastic bronchitis: An unusual bronchoscopic challenge associated with congenital heart disease repair.Int J Pediatr Otorhinolaryngol. 2003; 67: 543-548Crossref PubMed Scopus (22) Google Scholar Fenestration of the Fontan circuit was reported to be a therapeutic option, and we adopted a similar approach.5Wilson J Russell J Williams W et al.Fenestration of the Fontan circuit as treatment for plastic bronchitis.Pediatr Cardiol. 2005; 26: 717-719Crossref PubMed Scopus (51) Google Scholar The final therapeutic option for patients with failed Fontan physiology complicated by plastic bronchitis may be Fontan conversion and cardiac transplantation.6LaRue M Gossett JG Stewart RD et al.Plastic bronchitis in patients with Fontan physiology: Review of the literature and preliminary experience with Fontan conversion and cardiac transplantation.World J Pediatr Congenit Heart Surg. 2012; 3: 364-372Crossref PubMed Scopus (28) Google Scholar None. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI0MzQwNzcwMDRhNzAyMWZmYTk3ZTYxNTA4NzY3NzJkNiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjgxNjcxODkxfQ.F4k7kxLC3tA8HQAgfZx_e6lJAJi1gUZPTRjTKUlz1uMTmIMKqzINCl4DxAfzaRkSOqrz5LR2Tcb26igs_fByMQdRJMRMk0n5cCt0NRDLhJ7d24jfrXBiv04dvXgo6N4o2xBvLlMWdWLpRtIX8rNDuVKLlyuOWHX5z-UsCjkuyrNaRuM48IMHngZ1wXbijw6I_7j4GXMcC3Dm7gj_BhAiIJI4safemQXAN_Js7NI5rgaMZKbPdAPkGZ8RgXPG8Qun5dEedfmjJoFtpBE_A2r8GYX45PcpnlYHx8VqkwjLjE22NARydRiDUQZ1oSPizE0Ck-U1bVYExrGfMULzLGqh_Q Download .mp4 (0.91 MB) Help with .mp4 files Video 1. Bronchoscopy recording of the ball-like cast in the trachea. The Lymphatic System in the Fontan Patient—Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should KnowJournal of Cardiothoracic and Vascular AnesthesiaVol. 36Issue 8PreviewThe Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body’s immune response. Full-Text PDF