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Canadian Journal of Cardiology

Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies

Published:March 18, 2022DOI:https://doi.org/10.1016/j.cjca.2022.03.011

      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.

      Résumé

      La bronchite plastique (BP – aussi appelée maladie des moules bronchiques) et l’entéropathie exsudative (EE) sont des complications rares, mais potentiellement dévastatrices de l’intervention de Fontan. La BP touche environ 4 % des patients ayant subi cette intervention, généralement moins de deux à trois ans après la réalisation de celle-ci. Elle s’accompagne de toux chronique, de respiration sifflante, de fièvre ou d’asphyxie aiguë et se caractérise par la présence de moules bronchiques qui sont expectorés ou découverts au cours d’un examen bronchoscopique. L’EE touche de 4 à 13 % des patients, généralement moins de cinq à dix ans après qu’ils ont subi l’intervention de Fontan. Le tableau clinique associe œdème, ascite, hypoalbuminémie, lymphopénie, hypogammaglobulinémie et clairance fécale élevée d’alpha-1-antitrypsine. Ces troubles présentent des caractéristiques physiopathologiques similaires, impliquant une perturbation du système lymphatique attribuable à une pression veineuse centrale élevée sur fond de surproduction de lymphe et d’inflammation. S’ensuit le drainage de la lymphe dans des circuits à basse pression, tels que les bronches (BP) et le duodénum (EE). Notre compréhension de ces troubles s’est grandement améliorée au cours de la dernière décennie grâce aux progrès de l’imagerie du système lymphatique, notamment la lymphangiographie par résonance magnétique, et au succès précoce des interventions lymphatiques, dont l’embolisation lymphatique, l’embolisation du canal thoracique et la décompression percutanée du canal thoracique. La BP et l’EE nécessitent l’une et l’autre une prise en charge multidisciplinaire afin de traiter de façon optimale les lésions hémodynamiques résiduelles par cathétérisme, d’abaisser la pression veineuse centrale par un traitement médical, de limiter les symptômes et de cibler une perfusion lymphatique anormale lorsque les symptômes persistent. Dans le présent article de synthèse, nous résumons les caractéristiques physiopathologiques de la BP et de l’EE ainsi que les données probantes actuelles concernant la prise en charge de ces troubles. De plus, nous proposons des algorithmes de traitement, abordons les perspectives de recherche et traitons des facteurs clés à prendre en considération dans l’élaboration d’un programme de prise en charge interventionnelle des troubles lymphatiques.
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