Improved early results with cavopulmonary connections

Background: We describe the recent results in a large cohort of patients wi th functionally single ventricle who underwent various modifications of cav opulmonary connections. Methods: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations b etween June 1995 and June 1997. Demographic data, surgical history, and per ioperative course were reviewed. Results: We performed 130 consecutive oper ations in 113 patients. The procedures included superior cavopulmonary conn ections in the form of the HemiFontan procedure in 45 instances, and bidire ctional Glenn procedures in 11, and bilateral superior cavopulmonary connec tions in 7. The median age of these patients was 7.0 months. We completed F ontan operations using a fenestrated lateral tunnel on 47 occasions, and us ing an extracardiac conduit: 9 times, 5 of which were fenestrated. A latera l tunnel without fenestration was constructed in one patient. The median ag e for these procedures was 19.5 months. In the remaining 10 instances, we r evised Fontan procedures at a median age of 8 years. Diagnoses included hyp oplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of s urgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) becaus e of transient junctional rhythm, and 2 received treatment for supraventric ular tachycardia. Pleural effusions were diagnosed radiographically after 3 1 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in h ospital was 6 days, with a range from 3 to 58 days. Conclusion: Mortality a nd perioperative morbidity after cavopulmonary connections have decreased d ramatically in the current era. The long-term results of staged reconstruct ion for functionally single ventricle, nonetheless, await ongoing study.