Transconduit approach for pulmonary vein stenting in an extracardiac Fontan and plastic bronchitis case.

Plastic bronchitis (PB) is a rare but severe complication in patients following the Fontan operation, the final palliative procedure for single ventricle heart disease. PB is characterised by the formation of rubbery casts of the tracheobronchial tree that may cause cough, wheezing, dyspnoea and hypoxia. Progressive airway obstruction may lead to asphyxia and death, and the overall mortality rate is 15.2%. Most patients receive a combination therapy from three different treatment strategies, that is, therapy for relief of airway obstruction, anti-inflammatory treatment and treatment to improve haemodynamics of the Fontan physiology. Therapy improving haemodynamics consists of optimising the Fontan circulation or cardiac function, antiarrhythmic therapy, inhibition of lymph leakage/production, decompression of the Fontan circulation and heart transplantation.We present the case of a child with PB after extracardiac Fontan operation, who was refractory to balloon angioplasty and surgical release for pulmonary vein (PV) stenosis. We decided to perform stent implantation for the right PVs through transconduit puncture instead of fenestration because the position of fenestration was too high to access the PVs. After PV stenting via the transconduit approach, she experienced improvement in cough, wheezing and breathlessness, and a significant reduction in airway cast formation.

Fontan-associated plastic bronchitis waitlist and heart transplant outcomes: A PHTS analysis.

Plastic bronchitis is a rare post-Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis. All Fontan patients were identified in the PHTS database (2010 - 2018). Waitlist and post-transplant outcomes were compared between Fontan patients with and without plastic bronchitis. Competing outcomes and Kaplan-Meier analyses were used to assess the impact of plastic bronchitis on waitlist and post-transplant survival. A secondary analysis excluded those with PLE from the comparison cohort. Of 645 Fontan patients listed for heart transplant, 69 (11%) had plastic bronchitis. At listing, patients with plastic bronchitis were younger (8.9 vs 11.1 years, P = .02), but had few other differences in baseline characteristics. A fewer Fontan patients with plastic bronchitis were listed in the more recent era (46 [15.4%] in 2010-2014 vs 23 [6.6%] in 2015-2018, P < .01). Overall, there was no difference in waitlist (P = .30) or post-transplant (P = .66) survival for Fontan patients with and without plastic bronchitis. The results were similar after excluding patients with PLE. Contrary to prior reports, this relatively large series showed that plastic bronchitis did not have a negative impact on survival to or after heart transplantation in Fontan patients. Our study also found a 50% reduction in listing in the current era, which may indicate evolution in management of Fontan patients.

Successful Thoracic Duct Ligation for Plastic Bronchitis in an Adult.

Plastic bronchitis is a rare and potentially life-threatening disease characterized by the development of obstructive fibrinous tracheobronchial casts and hypoxic respiratory failure. With its poorly understood cause and rare occurrence in the adult population, few treatment strategies have been described in adults with this condition. In this report, we present a case of successful treatment of an adult with plastic bronchitis, using thoracic duct ligation and resulting in full resolution of airway cast development.

A Rare Case of Plastic Bronchitis in an Adult Patient After Cardiopulmonary Bypass.

Plastic bronchitis is a rare life-threatening complication observed after cardiopulmonary bypass (CPB). We describe a case of a 54-year-old man in whom a fulminant case of plastic bronchitis developed after coronary artery bypass grafting (CABG) and mitral valve repair. A brief review of the literature is also presented.

[The role of rigid bronchoscope in the diagnosis and treatment of plastic bronchitis].

OBJECTIVE: To explore the role of rigid bronchoscope in the diagnosis and treatment of plastic bronchitis. METHODS: Various clinical parameters, the key points of operation and postoperative recovery were analyzed in 8 patients with plastic bronchitis treated in Department and ICU using retrospective analysis. RESULTS: All cases demonstrated bronchial lumen stenosis, mucous membrance coarse, congestion, edema in the affected side. 2 cases had follicular hyperplasia, 1 case had a few ooze after taking out the plastic type. All cases had endogenous foreign bodies, taking the shape of the bronchial tree or a funicular. Block shape and sites were as follows: right main bronchus 2 cases, superior lobe of right lung 1 case, right middle bronchial 1 case, left main bronchus 2 cases, 1 case with left lower lobe, right main bronchus and left lower lobe bronchus 1 case. The breath sounds of the affected side become more enhanced after operation, with the alliviation of dyspnea. All cases recovered after ICU treatment. The pathologic examination were all type I plastic bronchitis. CONCLUSIONS: Removement of the endogenous foreign body via rigid bronchoscopy is the effective method in the treatment of plastic bronchitis. Plastic bronchitis is a rapid-developing critical, urgent disease.In order to reduce the mortality, early diagnosis and timely surgery are necessary.

Perioperative management of shock in two fontan patients with plastic bronchitis.

Plastic bronchitis is potentially a life-threatening complication of long-standing surgically palliated single ventricle congenital heart disease. Patients can present with hypoxia requiring urgent bronchoscopy for removal of bronchial casts. Perioperative care for these patients is challenging and anesthesia is associated with significant cardiac risk. As more surgically corrected single ventricle patients survive to adulthood, these patients are expected to present more frequently. This report details the perioperative management of 2 Fontan patients with hypoxia and significant plastic bronchitis disease burden.

IL-17 induces type V collagen overexpression and EMT via TGF-ß-dependent pathways in obliterative bronchiolitis.

Obliterative bronchiolitis (OB), a fibrotic airway lesion, is the leading cause of death after lung transplantation. Type V collagen [col(V)] overexpression and IL-17-mediated anti-col(V) immunity are key contributors to OB pathogenesis. Here, we report a previously undefined role of IL-17 in inducing col(V) overexpression, leading to epithelial mesenchymal transition (EMT) and subsequent OB. We observed IL-17-mediated induction of col(V) α1 chains [α1 (V)] in normal airway epithelial cells in vitro and detected α1 (V)-specific antibodies in bronchoalveolar lavage fluid of lung transplant patients. Overexpression of IL-17 and col(V) was detected in OB lesions in patient lung biopsies and in a murine OB model. IL-17 is shown to induce EMT, TGF-ß mRNA expression, and SMAD3 activation, whereas downregulating SMAD7 expression in vitro. Pharmacological inhibition of TGF-ßRI tyrosine kinase, p38 MAPK, or focal adhesion kinase prevented col(V) overexpression and EMT. In murine orthotopic lung transplants, neutralizing IL-17 significantly decreased TGF-ß mRNA and protein expression and prevented epithelial repair/OB. Our findings highlight a feed-forward loop between IL-17 and TGF-ß, leading to induction of col(V) and associated epithelial repair, thus providing one possible link between autoimmunity and OB after lung transplantation.

Successful management of plastic bronchitis in a child post Fontan: case report and literature review.

PURPOSE: Plastic bronchitis is the occlusion of the major bronchial airways by a firm, gelatinous mucoid cast. It is a rare condition, which while classically described in asthma and sickle cell disease has greater mortality in patients with congenital heart disease. The management of this disease is obscure given the lack of clinical data regarding treatment therapies. METHODS: We describe a case of an 11-year-old female status after Fontan surgery who presented with respiratory distress secondary to atelectasis of the right lung. RESULTS: A bronchoscopy was performed demonstrating an obstructing bronchial cast with successful extraction. The plastic bronchitis continued to recur and she was placed on multiple inhaled mucolytics as well as inhaled tissue plasminogen activator with temporary resolution. Further evaluation of the etiology of her casts revealed that she had elevated pulmonary arterial pressures. Repeated bronchoscopic removal of the casts was utilized as well as continuation of the aggressive airway clearance. Ultimately fenestration of her Fontan was performed along with treatment of pulmonary vasodilators sildenafil and bosentan. Although there was improvement of the cast formation, her airway clearance could only be weaned to four times a day therapy with which she was discharged home after a 3-month hospitalization. She continues to remain on this therapy and has not required hospitalization since the initial incident over 1 year ago. CONCLUSIONS: Plastic bronchitis in a patient with Fontan physiology presents a treatment dilemma that may require comprehensive therapy in severe cases such as described.

[Aspergillus tracheobronchitis in a lung transplant recipient]. / Traqueobronquitis aspergilar en paciente sometido a trasplante pulmonar.

BACKGROUND: Aspergillus tracheobronchitis is an uncommon cause of pulmonary aspergillosis and almost exclusively affects lung transplant recipients. There is no lung tissue involvement, thus the tracheobron-chial tree is only affected. Patients are asymptomatic, so it is important to make an early diagnosis to prevent progression of the infection and airway complications. Several prophylaxis and treatment strategies have proven to improve the prognosis. CLINICAL CASE: This is the case of a 56 year-old man who underwent bilateral lung transplant for chronic obstructive pulmonary disease (COPD) and developed Aspergillus tracheobronchitis. He received the usual prophylaxis with nebulized liposomal amphotericin B every 48 h. Routine bronchoscopy performed 2 weeks after transplantation showed inflammation with the presence of pseudomembranes that produced a 50% stenosis of the right bronchial anastomosis. Biopsy of the pseudomembranes and bronchial aspirate yielded Aspergillus fumigatus. The patient started treatment with voriconazole twice a day, bronchial debridement through bronchoscopy was carried out, and the treatment with nebulized liposomal amphotericin B was continued every other day. Ten weeks later, there were no endobronchial lesions and the bronchial aspirate cultures were negative. CONCLUSIONS: Aspergillus tracheobronchitis is a complication of the lung transplant recipient. Early diagnosis and prompt antifungal therapy, including new antifungal agents and local debridement, may significantly improve the outcome.

Treatment of plastic bronchitis by orthotopic heart transplantation.

This case illustrates the successful use of orthotopic heart transplantation for the treatment of plastic bronchitis in a 6-year-old boy with hypoplastic left heart syndrome, which developed 2 years after Fontan procedure. Transplantation was undertaken after he failed medical management of airway obstruction. He is currently 1-year post-cardiac transplantation and has no evidence of plastic bronchitis despite weaning of an aggressive airway clearance regimen.

Plastic bronchitis: resolution after heart transplantation.

Plastic bronchitis (PB) is a rare cause of obstructive airway disease in patients who have undergone partially corrective surgery for congenital heart disease (CHD). The etiology of plastic bronchitis in such patients is ill-defined, and treatment is ineffective. We report resolution of PB and severe obstructive airway disease after heart transplantation in a patient with CHD.

Management of plastic bronchitis in a child with mild intermittent asthma.

OBJECTIVES: We describe the clinical presentation of a 7 1/2-year-old boy with a history of mild intermittent asthma who presented with left lung collapse and was found to have plastic bronchitis. METHODS: We reviewed the patient chart and imaging results and performed a literature review of plastic bronchitis and its management. RESULTS: Bronchoscopy in our patient demonstrated a large white, friable, cast-like material that obstructed the entire left main stem bronchus and could not be easily suctioned. The cast was removed in a piecemeal fashion by means of serial rigid bronchoscopy over a 6-month period with use of both optical forceps and flexible suction catheters. Microscopic examination of the cast-like material showed a predominance of eosinophils along with neutrophils encased in proteinaceous material. CONCLUSIONS: Plastic bronchitis in children is a rare condition that can mimic foreign body aspiration and can be associated with underlying pulmonary inflammatory disorders or cardiovascular disease. Aggressive bronchoscopic management of the airway obstruction and medical management of the underlying disease process are important for the successful treatment of plastic bronchitis.

Aggressive bronchoscopic management of plastic bronchitis.

Plastic bronchitis or "Bronchitis Plastica" is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children's hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week's period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex.

Perioperative management of plastic bronchitis in children.

PURPOSE: The aim of this study was to summarize clinical features and perioperative management of plastic bronchitis in 21 children. METHODS: We reviewed the clinical data of 21 children diagnosed with plastic bronchitis in our hospital from April 2001 to February 2009 in this retrospective study. RESULTS: Bronchoscopy was performed in 19 of the 21 cases. Of these, 13 patients were cured with the first procedure, during which branching bronchial casts were taken out. Six cases required a second procedure; of these, five patients were cured by removing branching bronchial casts; one patient died from massive pulmonary hemorrhage during anesthesia induction. Two patients were critically ill at initial diagnosis and worsened despite airway intubation, these patients died of multiple organ failure. The branching bronchial casts which were composed primarily of gelatinous fibers were sent for pathologic examination. Pathologically, 12 foreign bodies were classified as type I casts (containing inflammatory cells), 9 cases were type II (no inflammatory cells detected). CONCLUSIONS: Plastic bronchitis in children is a life-threatening disease, the only effective therapeutic modality is bronchoscopic extraction. The early diagnosis of plastic bronchitis is difficult, anesthesia and extraction procedures are demanding, and the postoperative monitoring is challenging. The clinician should try to make an early diagnosis, use enhanced perioperative monitoring, and improve operational technique to provide timely treatment for the children with plastic bronchitis, thereby reducing the associated mortality rate.