Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation

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.

Plastic bronchitis due to adenoviral infection: a case report

“…Recently, other treatments of PB have mainly focused on percutaneous thoracic intervention after the Fontan procedure, including the administration of lowdose azithromycin, inhaled bronchodilators, and use of recombinant human DNase [18][19][20]. In addition, nebulization with N-acetylcysteine and biphasic cuirass ventilation have also been reported [21]. Similarly, in our case, hypoxemia was resolved after removal of the plastic casts.…”

Preduodenal Portal Vein Associated with Complex Congenital Heart Disease and Heterotaxy Syndrome: An Unexpected Cause of Common Complaint

“…Furthermore, making diagnosis for PDPV could become more difficult in patients with CHD because chest pain or abdominal discomfort could occasionally occur in association with their heart condition. 2 In the present cases, an initial diagnosis of CHD, a potentially life-threating condition, may lead to a delay in the diagnosis of PDPV. We should be aware of the possibility of PDPV when encountering a patient with HS who complains of common symptoms.…”

“…To identify PDPV as the cause of the common symptoms was challenging because they were confounded with those related to their CHD. 2 Case 1: A 12-year-old boy who had been diagnosed with complete atrioventricular septal defect, malposed great arteries, pulmonary atresia, bilateral superior caval veins, and right single coronary artery complained of general fatigue, headache, nausea, and postprandial abdominal discomfort. The patient was born at 41 weeks' gestational age, with a birth weight of 2868 g. He underwent placement of a modified Blalock-Taussig shunt at four months of age followed by bilateral bidirectional cavopulmonary shunt at seven months of age.…”

“…Repeated physical examinations, X-rays, and ultrasonography failed to reveal the causes of his symptoms. Because we suspected that the symptoms might be due to failing Fontan circulation, 2 we performed cardiac catheterization at 10 years of age. Central venous pressure, cardiac index, and pulmonary arterial resistance were 13 mmHg, 3.1 L/min/m 2 , 0.32 Wood units • m 2 , respectively.…”

Response to: Biphasic cuirass model

“…The concept of negative pressure ventilation precedes that of positive pressure ventilation and remains an intriguing possibility for the management of shared airway surgery in children. We were aware of the device and its use in pediatric critical care for the treatment of respiratory failure 1 and plastic bronchitis, 2 but are not aware of it being reported as a mechanism for maintaining ventilation during shared airway surgery in children, and as such did not include it as a recognized technique in our review.…”