The Science Journal of the American Association for Respiratory Care

2012 OPEN FORUM Abstracts

PLASTIC BRONCHITIS IN A PEDIATRIC PATIENT SECONDARY TO FONTAN PALLIATION.

Trent M. Tappan1, Gary R. Lowe1, Bhutta Adnan2; 1Respiratory Care Services, Arkansas ChildrenÂ’s Hospital, Little Rock, AR; 2Dept. of Pediatrics, Critical Care Medicine Section, University of Arkansas for Medical Sciences, Little Rock, AR

Introduction: Plastic Bronchitis (PB) is a condition where large casts or plugs are formed throughout the tracheobronchial tree causing obstruction of the airways which can lead to respiratory failure and possible cardiac arrest. This case describes the course of a 3 year old male who developed PB after undergoing a Fontan procedure. Case Summary: The patient underwent a fenestrated Fontan procedure and had to be taken back to surgery to address mediastinal bleeding. He was placed on VA ECMO post-operatively. After five days, he was weaned from VA ECMO support. He developed profound hypoxemia and was placed on VA ECMO a second time. A snare was placed on the fenestration between the right atrium and vena cava to control the blood flow through the pulmonary arteries. Pulmonary services performed a bronchoscopy and found a large cast, which extended throughout his entire tracheobronchial tree. It was later removed with a rigid bronchoscopy. Aggressive pulmonary toilet was started including acetylcysteine, dornase alfa, and Tissue Plasminogen Activator via nebulization, along with scheduled bronchoscopies. After 2 days, the patient was weaned from ECMO, but was unable to be weaned from positive pressure ventilation and underwent a tracheotomy. He seemed to improve when he developed another occlusive cast that resulted in cardiac arrest with asystole. A large tracheal cast was dislodged during chest compressions. The patient returned to his baseline level of consciousness. Due to the recurring tracheal casts, he was transferred to our institution where he received an orthotopic heart transplant and did not have any more occurrences of casts. Discussion: PB is also known as Fibrinous Bronchitis or Pseudo-Membranous Bronchitis and is an extremely rare and potentially fatal complication after Fontan procedure. It is characterized by expectoration of long, branching bronchial casts which can manifest in recurrent life threatening airway obstruction. The exact cause of the condition is unknown. Seear et al classifies this as type 2 (acellular) PB, consisting of casts composed of mucin with little or no cellular infiltrate and occurring only in children with congenital heart disease (CHD). The most common procedure associated with PB is the Fontan. This case demonstrates the severity of PB associated with CHD. It also illustrates that an orthotopic heart transplant along with aggressive pulmonary toilet can lead to the survival of patients with reoccurring PB. Sponsored Research - None