A Multifaceted Approach to the Management of Plastic Bronchitis After Cavopulmonary Palliation

Abstract

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 Retrospective case series of medical, surgical, and catheter-based management of patients with plastic bronchitis after cavopulmonary palliation. Results Fourteen patients (86% male, 93% white) were included. Median age at Fontan operation was 2.7 years (range 1.2–4.1) with median interval to plastic bronchitis presentation of 1.5 years (9 days–15.4 years). Cast composition was available for 11 patients (79%) and included fibrin deposits in 7. All patients were treated with pulmonary vasodilators. Thirteen patients (93%) were treated with inhaled t-PA. Hemodynamically significant lesions in the Fontan pathway were addressed via catheter-based (n=9) and surgical (n=3) interventions. Three patients (21%) underwent heart transplantation. Median length of follow-up was 2.7 years (0.6–8.7). Symptoms improved such that six of thirteen (46%) patients were weaned off t-PA. Rare or episodic casts are successfully managed with outpatient t-PA in the majority of other patients. Of the 3 patients who underwent heart transplant, two are asymptomatic and one has recurrent casts in the setting of elevated filling pressures and rejection. Conclusions A systematic step-wise algorithm, which includes optimization of hemodynamics, aggressive pulmonary vasodilation and inhaled t-PA, is an effective treatment strategy for patients with plastic bronchitis after cavopulmonary connection.

The Relentless Effects of the Fontan Paradox

“…16 The symptoms of plastic bronchitis can be mitigated by using aerosolized tissue plasminogen activator, which acts as an airway clearing agent by effectively breaking down cast material that can then be more easily passed. 17 Aggressive treatment with pulmonary vasodilator agents such as sildenafil or bosentan may theoretically lower impedance to forward passive cavo-pulmonary flow, thereby placing the circulation at a point just beneath the threshold for protein spillage and has been an important component of the treatment strategy for both plastic bronchitis and PLE.…”

Patients With Systemic Right Ventricle Are at Higher Risk of Chylothorax After Cavopulmonary Connections

“…It is now understood that the capacity of a patient's lymphatic circulation to drain the increased production of lymph after Fontan completion is determining some of their outcomes [9]. Patients with protein-losing enteropathy and plastic bronchitis have been found to have inadequate lymphatic drainage [16,17]. It has been suggested that patients' lymphatic circulation could be categorized before Fontan completion, and those patients with an already engorged lymphatic circulation would have worse outcomes.…”

Efficacy of fiberoptic bronchoscopy and bronchoalveolar lavage in childhood‐onset, complicated plastic bronchitis

“…The true prevalence of PB is unknown, as it is likely that many patients with mild forms of the disease are undiagnosed. 16,25 Many clinicians are unfamiliar with the disease and may fail to recognize milder forms of the syndrome. 3 In the current study, during admission, only two children had tachycardia, and all other cases showed no signs or symptoms of hypoxia, suggesting that they might be milder forms of PB.…”