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Bronchoscopy for specific situations
Published in Don Hayes, Kara D. Meister, Pediatric Bronchoscopy for Clinicians, 2023
Douglas Sidell, Christopher T. Towe, MyMy C. Buu
Plastic bronchitis is a condition leading to formed branching casts filling the airways (Figure 9.10). It is associated with cardiac abnormalities, cystic fibrosis, asthma, sickle cell anemia, lymphagiomatosis, and can occur idiopathically. It is most commonly seen in patients with single-ventricle heart disease after palliation. The casts can be made up of protein, fibrin, mucin, or inflammatory cells. Obstruction of the airway leads to hypoxemia, cough, and respiratory distress. Treatment includes bronchoscopy for cast removal and medical management with inhaled medications and chest physiotherapy.17
Plastic bronchitis
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
Plastic bronchitis is a rare disease that mainly occurs in children and is characterized by the expectoration or bronchoscopic removal of complex, branching bronchial casts. Unlike other diseases with mucous plugging such as allergic bronchopulmonary aspergillosis (ABPA) and asthma, these casts are large, rubbery, and branching on gross inspection and acellular on histopathology. The disease may be related to abnormalities in lymphatic drainage. Frequently patients with plastic bronchitis have an underlying systemic illness, with congenital heart disease, particularly after corrective surgery, being the most common (Dori et al., 2014). Patients present with cough, dyspnea, and airway obstruction and are often misdiagnosed initially as having reactive airway disease or pneumonia. Standard treatment for reactive airway disease and mucous plugging with albuterol, steroids, acetylcysteine, and dornase alfa are typically ineffective. Other therapies such as tissue plasminogen activator, and pulmonary vasodilators, such as sildenafil, have been used. Thoracic duct ligation and selective lymphatic collateral embolization have been shown to be curative (Caruthers et al., 2013). Acute bronchoscopic removal of casts causing airway obstruction may be lifesaving.
Interventions in Congenital Heart Disease:A Review of Recent Developments: Part I
Published in Structural Heart, 2021
Patients with single ventricle morphology having Fontan palliation have a high mortality and are susceptible to a wide range of acute and late morbidities.80,81 Specifically, the development of protein-losing enteropathy (PLE) or plastic bronchitis (PB) confers a poor prognosis, with 5-year survival ranging from 46% in early studies to 70 to 88% in the current era.80,82 The etiology of PLE and PB is incompletely understood, but fundamentally related to chronically elevated central venous pressure. These altered hemodynamics result in a combination of increased lymphatic production and impedance to lymphatic drainage, resulting in elevated lymphatic pressures and eventually leakage or retrograde flow of lymphatic fluid into low-pressure lumens such as the gut and bronchi.83 Medical management of this insidious and often progressive disease remains challenging.
Current evidence for pharmacologic therapy following stage 1 palliation for single ventricle congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2022
Meredith C. G. Broberg, Ira M Cheifetz, Sarah T Plummer
Beta-blockers are also used in pediatric heart failure, however with less robust evidence to support their use. Shaddy et al. in a multicenter, randomized control trial [48] observed no difference between treatment groups (low dose carvedilol, high dose carvedilol, or placebo) in the percent of pediatric patients with decreased ventricular function who improved, worsened, or were unchanged. This study included a heterogeneous study population including patients with pediatric cardiomyopathy and congenital heart disease (including single ventricle disease). Only examining patients with systemic left ventricles demonstrated a trend for improvement. A non-beneficial trend was seen in those patients without a systemic left ventricle. Miyamoto et al. studied the right ventricle (RV) of explanted hearts from children with HLHS [49]. They included three cohorts: 1) patients undergoing elective transplants (younger cohort without symptoms of heart failure) 2) patients listed for transplant after failing surgical palliation with right ventricular failure and/or refractory protein-losing enteropathy or plastic bronchitis and 3) non-failing controls from structurally normal hearts. Similar to adult heart failure models altered gene expression was seen. There was down regulation of beta 1 adrenergic receptors in all HLHS RVs compared to non-failing RVs. However, key differences were discovered in downstream gene expression when comparing the decompensated HLHS RV to adult heart failure models. These differences, which lead to increased sarcoplasmic reticulum calcium content in decompensated HLHS RV, may offer an explanation as to why the carvedilol trial suggested differences in efficacy of blocker therapy with ventricular morphology.
An update on current and emerging treatments for pulmonary arterial hypertension in childhood and adolescence
Published in Expert Review of Respiratory Medicine, 2019
Julie Wacker, Robert Weintraub, Maurice Beghetti
An elevation of the mPAP in patients with a single ventricle physiology palliated with a Fontan, although not fulfilling PH criteria, can lead to Fontan failure. Risk factors for Fontan failure, protein-losing enteropathy, and plastic bronchitis are a mean PAP >15 mmHg, a transpulmonary gradient >8 mmHg, and PVRi >2.5 WU·m2. A few studies have suggested an improvement in exercise capacity and symptoms with PDE-5 inhibitors [80] and ERA [81,82], and a clinical trial is ongoing (NCT02080637).