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Background . Chylopericardium is a rare complication after operation for congenital heart disease. The incidence and clinical outcomes in a large cohort of surgical patients are unknown. Methods . We retrospectively reviewed the clinical records spanning more than 12 years in a single institution of 16 children with chylopericardium after cardiac operation. Results . We identified 16 patients with chylopericardium between 1985 and 1997. Chylopericardium was isolated in 7 patients. Twelve patients required pericardial drainage. Patients with isolated chylopericardium presented late and were treated initially as having postpericardiotomy syndrome. Three patients underwent thoracic duct ligation. There were two late deaths unrelated to the chylothorax. Associated diagnoses were internal jugular vein thrombosis and recurrent pulmonary vein obstruction (1 of 16 patients), an associated syndrome but not Turner or Noonan (10 of 16), superior cavopulmonary or total cavopulmonary anastomosis (7 of 16), atrioventricular septal defect repair (5 of 16), and repair of tetralogy of Fallot (2 of 16). Conclusions . Percutaneous drainage to relieve tamponade together with a low-fat or medium-chain triglyceride diet results in resolution in most cases of postoperative chylopericardium. If a pericardial effusion enlarges, fails to clear on aspirin therapy, or presents late after hospital discharge, diagnostic pericardial tap and a low-fat diet are indicated.

Background . There is a paucity of information to guide the management of the child with Down's syndrome and congenital heart disease in whom biventricular repair is precluded. Methods . Through the cardiology and cardiovascular surgery databases of The Hospital for Sick Children and Toronto Congenital Cardiac Centre for Adults, we identified patients with trisomy 21 and ventricular hypoplasia who had undergone a Fontan procedure (or modification). Results . Of 533 patients who had undergone a Fontan operation between 1976 and 1997, 4 had trisomy 21. All 4 patients had unbalanced complete atrioventricular septal defect with right ventricular hypoplasia in 3 and left ventricular hypoplasia in 1. Three patients survived, and 1 died of endocarditis. The 3 survivors have done well in the short term and medium term without complications related to the pulmonary vasculature. Conclusions . We suggest that in appropriately selected patients with trisomy 21 and ventricular hypoplasia who are unsuitable for two or one and a half ventricle repair, the Fontan procedure is not contraindicated and provides short-term and medium-term benefit.

Objectives: We have shown that exhaled nitric oxide levels decrease after surgical closure of congenital left-to-right cardiac shunts. It remains unclear whether the change in exhaled nitric oxide levels reflects endothelial injury caused by the use of cardiopulmonary bypass or the decrease in pulmonary blood flow attendant on shunt closure. Transcatheter atrial septal defect closure permits shunt closure without the use of cardiopulmonary bypass. Therefore we compared changes in exhaled nitric oxide levels after surgical and transcatheter device closure of atrial septal defects. Methods: We enrolled sequentially 30 children undergoing atrial septal defect closure. Fifteen patients (age range, 0.4-16 years; median age, 6.5 years) underwent surgical atrial septal defect closure with cardiopulmonary bypass, and 15 patients (age range, 4-17 years; median age, 8.4 years) had device closure of the atrial septal defect in the catheterization laboratory. We measured nitric oxide levels in end-tidal expiratory gas with a rapid-response chemiluminescent analyzer before and after atrial septal defect closure. Results: After surgical repair of the atrial septal defect, exhaled nitric oxide decreased by 21%, from 10.9 ± 4.4 to 8.4 ± 3.3 ppb (P