Search

Fibrinous bronchitis, also known as plastic bronchitis or pseudomembranous bronchitis, usually manifests clinically with non-productive cough and dyspnea. Prior to the expectoration of plaque formations, patients have a history of viral or other respiratory disease. Plastic bronchitis is an extremely rare and fatal complication associated with Fontan procedure. The condition is characterized by the formation of inspissated bronchial casts which may cause life-threatening airway obstructions. Although the pathogenesis of this condition remains unclear, it is believed to involve elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage. A 9-year-old male patient with Down syndrome underwent Fontan procedure 29 months earlier due to complete endocardial cushion defect and single ventricular physiology. The patient presented to the emergency department due to sudden-onset respiratory distress and fever (38.5 °C). Bilateral diffuse sibilant rhonchi, secretory rales, and intercostal retractions were noted during pulmonary system examination. Posterior-anterior chest x-ray showed cardiomegaly (cardiothoracic ratio=0.6) and bilateral diffuse infiltration. While under treatment, the patient experienced sudden-onset cough with expectoration of rubbery sputum in the form of branching bronchi-shaped casts. Samples of the inspissated sputum were sent to the Pathology and Microbiology departments for examination. The pathology report indicated fibrinoid material composed of a small number of inflammatory cells and bacterial plaques. Normal bacterial flora was identified in microbiological culture. Clinical presentation can vary from mild clinical findings to life-threatening symptoms. As in our patient, the diagnosis is made clinically, based on expectoration of bronchial casts or their detection during bronchoscopy. As our patient responded well to medical treatment, we proceeded with clinical follow-up. Plastic bronchitis is a very rare entity, and carries a poorer prognosis when it develops after congenital heart disease, as in our case.

Objective: In this study, we report the current indications, early-midterm results and the outcome of pulmonary artery banding (PAB) operation after definitive repair. Methods: Between 2000 and 2007, 28 infants underwent PAB operation. Ages were between 21 days and 6 months (mean 3.0±1.7 months). All patients had pulmonary hypertension at systemic level. Fourteen patients were candidates for terminal biventricular repair [atrioventricular septal defect (AVSD) (n=6), double outlet right ventricle (DORV)+ ventricular septal defect (VSD) (n=4), Swiss cheese VSD (n=2), transposition of the great arteries (TGA)+Swiss cheese VSD (n=1), ASD+VSD+aortic coarctation (n=1)], and the remaining 14 were candidates for terminal univentricular repair [double inlet left ventricle (n=5), double inlet right ventricle (n=2), AVSD+left ventricular (LV) hypoplasia (n=2), DORV+LV hypoplasia (n=2); tricuspid atresia (n=1), left AV valve atresia (n=1), TGA+Swiss cheese VSD+LV hypoplasia (n=1). Results: Three patients (1 in biventricular group; 2 in univentricular group) died in the early postoperative period (10.7%). Three patients needed long duration of mechanical ventilatory support. Twenty- three of the surviving patients (92%) were followed -up between 1 month to 7 years. Currently 7 patients underwent successful biventricular repair and five patients underwent univentricular repair (extracardiac Fontan 3; Glenn 2). Two patients died early and 1 patient died 1 year after extracardiac Fontan operation. Survival for biventricular group was 92.8% at 1 and 4 years and 85.7% and 58.4% respectively for univentricular group (p<0.05) Conclusion: Pulmonary artery banding operation has still a significant role in the palliation of certain congenital cardiac anomalies. Outcome of patients who are candidates for biventricular repair is better than the univentricular repairs. [ABSTRACT FROM AUTHOR]