ABSTRACT. Plastic bronchitis is a condition in which large, bronchial casts with rubber-like consistency develop in the tracheobronchial tree and cause airway obstruction. We describe a 4-year-old girl who had Fontan physiology and who developed plastic bronchitis and report for the first time the use of aerosolized tissue plasminogen activator for treatment of this condition. The literature is reviewed with emphasis placed on the occurrence of this disorder in patients with single ventricle physiology. Pediatrics 2002;109(4). URL: http:// www.pediatrics.org/cgi/content/full/109/4/e67; plastic bronchitis, bronchial cast, Fontan, congenital heart disease, tissue plasminogen activator.ABBREVIATION. t-PA, tissue plasminogen activator. P lastic bronchitis is a condition in which large, pale tan, bronchial casts with rubber-like consistency develop in the tracheobronchial tree and cause airway obstruction. 1 It is an unusual disorder that occurs in various disease states, and fewer than a dozen cases have been reported regarding patients with palliated congenital heart disease. We describe a 4-year-old girl who had Fontan physiology and who developed plastic bronchitis and report for the first time the use of aerosolized tissue plasminogen activator (t-PA) for treatment of this condition. The literature is reviewed with emphasis placed on the occurrence of this disorder in patients with single ventricle physiology. CASE REPORTOur patient's double-inlet left ventricle and L-transposition of the great arteries were diagnosed at birth. Her cardiac palliation performed at an outside institution consisted of a pulmonary artery band at 1 month of age; a bidirectional Glenn operation at 12 months; and a fenestrated, lateral tunnel Fontan operation at 26 months. Fenestration closure using a subcutaneous snare was performed 6 months later. Cardiac catheterization at 48 months of age revealed excellent hemodynamics without Fontan pathway obstruction. The mean pressure in the lateral tunnel was 10 mmHg, and there was no gradient to the left or right pulmonary arteries. The left ventricular end diastolic pressure was 4 mmHg, and the bulboventricular foramen was unobstructed.At 53 months of age, she presented to our institution with respiratory failure requiring mechanical ventilation. She improved with nebulized bronchodilator therapy and chest physiotherapy. This episode of respiratory decompensation was attributed to a mucus plug. During the next 4 months, she had 4 additional hospital admissions for perceived asthma exacerbations and pneumonia. Chest radiographs on each of these admissions revealed shifting infiltrates and atelectasis. A bronchoscopy performed during the third admission was unremarkable. The diagnosis of plastic bronchitis was made when the patient's parent brought an expectorated bronchial cast to the pediatrician after the fourth admission and bronchoscopy during a subsequent admission revealed a large bronchial cast. She was then treated on an outpatient basis with chest physiotherapy, inhaled steroids, al...

Objective To investigate the possible effects of antiretroviral therapy (ART) in utero on cardiac development and function in HIV-negative children. Background ART reduces vertical HIV transmission. Long-term cardiotoxicity after in utero exposure to ART is unknown in children but has occurred in young animals. Methods Using a prospective multi-site cohort study design, we compared echocardiograms taken between birth and 24 months in two groups of HIV-negative infants of HIV-positive mothers: 136 infants exposed to ART (ART+) and 216 unexposed infants (ART−). Results Mean LV mass Z-scores were consistently lower in ART+ girls than in ART− girls: differences in mean Z-scores were −0.46 at birth (P=0.005), −1.02 at 6 months (P<0.001), −0.74 at 12 months (P<0.001), and −0.79 at 24 months (P<0.001). Corresponding differences in Z-scores for boys were smaller: 0.13 at 1 month (P=0.42), −0.44 at 6 months (P=0.01), −0.15 at 12 months (P=0.37), and −0.21 at 24 months (P=0.21). Septal wall thickness and LV dimension were smaller than expected in ART+ infants, but LV contractility was consistently about 1 SD higher at all ages (P<0.001). In ART+ infants, LV fractional shortening was higher than in ART− infants; girls showed a greater difference. Conclusion Fetal exposure to ART is associated with reduced LV mass, LV dimension, and septal wall thickness Z-scores and increased LV fractional shortening and contractility up to age 2 years. These effects are more pronounced in girls than in boys. Fetal ART exposure may impair myocardial growth while improving depressed LV function.

When a new left atrial mass is discovered by means of echocardiography, the differential diagnosis is usually between thrombus, vegetation, and tumor. 1 Moreover, several steps may be necessary to establish an accurate diagnosis. Therapy may be different for each of these conditions and may include anticoagulation, antibiotics, or operation. Recently, however, we were able to document another cause that allowed us to avoid an unnecessary reoperation or therapeutic intervention.