A Case of Plastic Bronchitis Presenting 9 Years After Fontan

Abstract

A 15-year-old boy with hypoplastic left heart syndrome experienced plastic bronchitis 9 years after completion of a nonfenestrated lateral tunnel Fontan. Despite cardiac catheterization with coil embolization of collateral vessels and initiation of a pulmonary toilet regimen, including aerosolized tissue plasminogen activator, he continued to expectorate large acellular-mucinous casts. Finally, after optimization of cardiac function with the addition of carvedilol, the expectorated casts decreased in number. This report reviews pathophysiology of plastic bronchitis cast formation and therapy in the context of this late presentation after Fontan.

Prospective, Longitudinal Study of Plastic Bronchitis Cast Pathology and Responsiveness to Tissue Plasminogen Activator

“…At present, inhaled tPA is anecdotally used to treat PB [7, 9, 28, 29]. In this study we showed that tPA, in amounts derived from experimental models, was effective in reducing cast mass ex vivo [10, 15, 25].…”

“…Plastic bronchitis (PB) is a rare, primarily pediatric disease that may be a complication of congenital heart disease (CHD) or its surgical palliation by the Fontan procedure [7, 9, 12, 13, 16, 21, 29]. The hallmark of PB, is the formation of rubbery “casts” in the airway (figure 1) that have been reported to be comprised of mucin, fibrin or a mixture of both and can partially or completely obstruct the airways [19, 22].…”

“…In some cases, fibrinolytic agents, inhaled urokinase or tissue plasminogen activator (tPA), both with unknown clinical safety and efficacy, have been used to reduce cast burden [7, 9, 22, 28, 29]. At the University of Michigan, inhaled tPA (Activase ® , Genentech, S. San Francisco, CA USA) is commonly used in repeated doses for the treatment of acute PB.…”

Aggressive bronchoscopic management of plastic bronchitis

“…Children with underlying cardiovascular disease are at risk of developing this condition [1][2][3][4][5]. We present three cases of children with plastic bronchitis after having undergone a Fontan procedure for hypoplastic left ventricle syndrome.…”

Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall

“…First, a long delay in presenting symptoms of PB after Fontan surgery is unusual [5], as the onset of PB is typically within 1 to 3 years after surgical operation. This finding alerts to regularly evaluate cardiac function and watch over the symptoms of PB in patients who received Fontan intervention.…”