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

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@article{f3c867d4f36b45f397630c9ce459c578,
title = "Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies",
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.",
keywords = "Bronchitis/diagnosis, Fontan Procedure/adverse effects, Heart Defects, Congenital/complications, Humans, Plastics, Protein-Losing Enteropathies/diagnosis",
author = "Mackie, {Andrew S} and Veldtman, {Gruschen R} and Lene Thorup and Hjortdal, {Vibeke E} and Yoav Dori",
note = "Copyright {\textcopyright} 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.",
year = "2022",
month = jul,
doi = "10.1016/j.cjca.2022.03.011",
language = "English",
volume = "38",
pages = "988--1001",
journal = "Canadian Journal of Cardiology",
issn = "0828-282X",
publisher = "Pulsus Group Inc",
number = "7",

}

RIS

TY - JOUR

T1 - Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient

T2 - Evolving Understanding and Emerging Therapies

AU - Mackie, Andrew S

AU - Veldtman, Gruschen R

AU - Thorup, Lene

AU - Hjortdal, Vibeke E

AU - Dori, Yoav

N1 - Copyright © 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

PY - 2022/7

Y1 - 2022/7

N2 - 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.

AB - 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.

KW - Bronchitis/diagnosis

KW - Fontan Procedure/adverse effects

KW - Heart Defects, Congenital/complications

KW - Humans

KW - Plastics

KW - Protein-Losing Enteropathies/diagnosis

UR - http://www.scopus.com/inward/record.url?scp=85130905996&partnerID=8YFLogxK

U2 - 10.1016/j.cjca.2022.03.011

DO - 10.1016/j.cjca.2022.03.011

M3 - Review

C2 - 35314335

VL - 38

SP - 988

EP - 1001

JO - Canadian Journal of Cardiology

JF - Canadian Journal of Cardiology

SN - 0828-282X

IS - 7

ER -

ID: 79722191