Abstract
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.
Keywords:
Central venous pressure; Congenital heart disease; Continuous negative extrathoracic pressure; Functionally univentricular circulation; Low cardiac output.
MeSH terms
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Adolescent
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Albumins / therapeutic use*
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Anticoagulants / therapeutic use
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Bronchitis / diagnosis
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Bronchitis / etiology
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Bronchitis / physiopathology
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Bronchitis / therapy
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Cardiac Output, Low / diagnosis
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Cardiac Output, Low / etiology
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Central Venous Pressure
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Fontan Procedure / adverse effects*
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Fontan Procedure / methods
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Heparin / therapeutic use*
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Humans
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Hypoplastic Left Heart Syndrome* / physiopathology
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Hypoplastic Left Heart Syndrome* / surgery
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Male
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Mineralocorticoid Receptor Antagonists / therapeutic use*
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Postoperative Complications* / diagnosis
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Postoperative Complications* / physiopathology
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Postoperative Complications* / therapy
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Protein-Losing Enteropathies* / diagnosis
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Protein-Losing Enteropathies* / etiology
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Protein-Losing Enteropathies* / physiopathology
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Protein-Losing Enteropathies* / therapy
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Respiration, Artificial / methods*
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Treatment Outcome
Substances
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Albumins
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Anticoagulants
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Mineralocorticoid Receptor Antagonists
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Heparin