Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies |
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| Affiliation: | 1. Division of Cardiology, Stollery Children’s Hospital and University of Alberta, Edmonton, Alberta, Canada;2. Scottish Adult Congenital Cardiac Service, Golden Jubilee Hospital and Institute of Cardiovascular and Medical Sciences, University of Glasgow, Glasgow, Scotland;3. Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen, Denmark;4. Division of Cardiology, Children’s Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA;1. Department of Pediatrics (Neurology), The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada;2. Department of Pediatrics (Neurology), BC Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada;3. Division of Neurology, Vancouver Stroke Program, University of British Columbia, Vancouver, British Columbia, Canada;1. Department of Psychiatry and Behavioural Neurosciences, McMaster University, Hamilton, Ontario, Canada;2. School of Rehabilitation Science, McMaster University, Hamilton, Ontario, Canada;3. Child Health Evaluative Science, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada;4. Factor-Inwentash Faculty of Social Work, University of Toronto, Toronto, Ontario, Canada;5. Transplant and Regenerative Medicine Centre, The Hospital for Sick Children, Toronto, Ontario, Canada;1. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitari i Politecnic La Fe, Instituto de Investigación Sanitaria La Fe, Centro de Investigación Biomédica en Red Cardiovascular, Valencia, Spain;2. Unitat Integrada de Cardiopaties Congènites de l’Adolescent i l’Adult Vall d’Hebron-Sant Pau, Department of Cardiology, Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain;3. Centro de Investigación Biomédica en Red Cardiovascular, Barcelona, Spain;4. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario La Paz, Madrid, Spain;5. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla, Centro de Investigación Biomédica en Red Cardiovascular, Sevilla, Spain;6. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital General Universitario Gregorio Marañon, Centro de Investigación Biomédica en Red Cardiovascular, Madrid, Spain;7. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario 12 Octubre, Madrid, Spain;8. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Clinic Barcelona, Institut Clinic Cardiovascular, Instituto de Investigaciones Biomédicas August Pi i Sunyer, Centro de Investigación Biomédica en Red Cardiovascular, Barcelona, Spain;1. Stollery Children’s Hospital, Edmonton, Alberta, Canada;2. The Hospital for Sick Children, Toronto, Ontario, Canada;3. Texas Children’s Hospital, Houston, Texas, USA;4. BC Children’s Hospital, Vancouver, British Columbia, Canada;5. Mazankowski Heart Institute, University of Alberta, Edmonton, Alberta, Canada |
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| Abstract: | Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ～4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted. |
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