Original article
Congenital heart surgery
A Multifaceted Approach to the Management of Plastic Bronchitis After Cavopulmonary Palliation

https://doi.org/10.1016/j.athoracsur.2014.04.015Get rights and content

Background

Plastic bronchitis is a rare, potentially life-threatening complication after Fontan operation. Hemodynamic alterations (elevated central venous pressure and low cardiac output) likely contribute to the formation of tracheobronchial casts composed of inflammatory debris, mucin, and fibrin. Pathologic studies of cast composition support medical treatment with fibrinolytics such as inhaled tissue plasminogen activator (t-PA).

Methods

This was a retrospective case series of medical, surgical, and catheter-based treatment of patients with plastic bronchitis after cavopulmonary palliation.

Results

Included were 14 patients (86% male, 93% white). Median age at Fontan operation was 2.7 years (range, 1.2 to 4.1 years), with median interval to plastic bronchitis presentation of 1.5 years (range, 9 days to 15.4 years). Cast composition was available for 11 patients (79%) and included fibrin deposits in 7. All patients were treated with pulmonary vasodilators, and 13 (93%) were treated with inhaled t-PA. Hemodynamically significant lesions in the Fontan pathway were addressed by catheter-based (n = 9) and surgical (n = 3) interventions. Three patients (21%) underwent heart transplantation. Median follow-up was 2.7 years (range, 0.6 to 8.7 years). Symptoms improved, such that 6 of 13 patients (46%) were weaned off t-PA. Rare or episodic casts are successfully managed with outpatient t-PA in most of the other patients. Of the 3 patients who underwent heart transplant, 2 are asymptomatic and 1 has recurrent casts in the setting of elevated filling pressures and rejection.

Conclusions

A systematic step-wise algorithm that includes optimization of hemodynamics, aggressive pulmonary vasodilation, and inhaled t-PA is an effective treatment strategy for patients with plastic bronchitis after cavopulmonary connection.

Section snippets

Patients and Methods

All patients with a clinical or pathologic diagnosis of plastic bronchitis managed through the Single Ventricle Survivorship Program at the Children's Hospital of Philadelphia between January 1, 2003, and August 22, 2013, were eligible for inclusion in this retrospective case series. Hospital records were reviewed for demographic information and pertinent clinical characteristics. Demographic and anatomic characteristics were summarized by standard descriptive summaries and are expressed as

Results

Fourteen patients met the inclusion criteria. Demographic characteristics are summarized in Table 1. Patients were 86% male and 93% white. Hypoplastic left heart syndrome was present in 6 patients (43%). Thirteen patients underwent Fontan completion at median age of 2.7 years (range, 1.2 to 4.1 years), with an extracardiac conduit in 6 patients (46%), lateral tunnel in 6 (46%), and a catheter-based covered stent baffle (performed at an another institution) in 1 (8%). One patient was not deemed

Comment

Our experience demonstrates that plastic bronchitis after Fontan operation can be successfully managed with complete elimination or a substantial reduction in symptoms. This is achieved through a step-wise, individually tailored strategy aimed at improving Fontan circulation through pharmacologic, catheter-based, or surgical treatments, or a combination of these, along with aggressive use of aerosolized t-PA to maintain airway patency (Table 3).

Plastic bronchitis is a serious, potentially

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