Excerpt

A 6-year-old girl was born with cyanotic congenital heart disease (double-outlet right ventricle, unbalanced atrioventricular septal defect, hypoplastic left ventricle, and pulmonary valve atresia) and underwent right modified Blalock-Taussig shunt inserted at 2 weeks of age. Cardiac catheterization (CaCh) at 6 months of age demonstrated that the branch pulmonary arteries were of good size; systemic ventricular pressure was 83/12 mmHg (albeit after the patient had received fluid and contrast); and multiple systemic-pulmonary collaterals had been embolized using coils. The patient subsequently underwent bidirectional cavopulmonary anastomosis. She was admitted at 13 months of age with a wet cough, further decrease in oxygen saturation, and rhinorrhea. Shortly after discharge, she expectorated two white bronchial casts, one of which clearly resembled a bronchial tree and was approximately 3 cm long. Pathologic examination of one of the casts revealed mucoid secretions, many small mononuclear cells, no neutrophils, and rare eosinophils, and cultures were negative. A subsequent CaCh at 15 months of age demonstrated good-sized branch pulmonary arteries, unobstructed bidirectional cavopulmonary anastomosis, good systemic ventricular function with end-diastolic pressure (SVEDP) 6 mmHg, and transpulmonary gradient (TpG) 5 mmHg. The patient continued to expectorate casts despite high-dose fluticasone. Changing her therapy to nebulized heparin, 155 U/kg/dose, administered twice daily modestly decreased her cast production without altering her coagulation profile or bone mineral density. …