We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26 ± 1.4 to 39 ± 4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.

original article:
https://www.researchgate.net/publication/325303179_Successful_Treatment_of_Protein-Losing_Enteropathy_and_Plastic_Bronchitis_by_Biphasic_Cuirass_Ventilation_in_a_Patient_with_Failing_Fontan_Circulation

authors:
Seigo Okada,1PhD, Jun Muneuchi,1PhD, Yusaku Nagatomo,1MD, Kaori Nonaka,2MSc,Chiaki Iida,1MD, Hiromitsu Shirouzu,1MD, Ryohei Matsuoka,1MD,Mamie Watanabe,1MD and Kunitaka Joo,1MD

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