Transcatheter closure of atrial septal defects in children with a hypoplastic right ventricle.

The efficacy and safety of device closure of atrial defects in children with complex congenital heart disease and a hypoplastic right ventricle have not been detailed. The objective of this study was to determine the clinical impact and outcomes of a staged surgical catheter-based management strategy. A retrospective analysis of 17 children with a hypoplastic right ventricle who had undergone cardiac catheterization and attempted device occlusion of an atrial defect was undertaken. Clinical data, anatomical diagnoses, previous surgeries, and interventions were noted. The clinical course and echocardiographic findings were compared before and after defect closure (6 +/- 4 months of follow-up). Nine children had pulmonary atresia with an intact ventricular septum, six had Ebstein's anomaly of the tricuspid valve, and two had isolated right ventricular hypoplasia. The mean age at defect closure was 6.6 +/- 3.4 years. Under general anesthesia, closure of the atrial defect was undertaken after initial temporary test occlusion. The Qp:Qs ratio before closure was 1.1 +/- 0.4. Oxygen saturation improved in all children from 91% +/- 1% to 98% +/- 1% (P < 0.0005). Mean right atrial pressure increased from 9 +/- 3 to 11 +/- 4 mm Hg after closure, but the difference was not statistically significant (P = 0.2). Follow-up revealed an increased right ventricular length (P = 0.009) and Z-score (P = 0.02), although no significant increase in the diameter of the tricuspid valve annulus was observed in children with pulmonary atresia. Right ventricular systolic pressures or systolic function did not change significantly during follow-up (P = 0.5 and 0.29, respectively). Exercise tolerance improved in all children and clinical evidence of right ventricular decompensation was absent. Catheter closure of atrial defects in children with a hypoplastic right ventricle is well tolerated, safe, and effective as a treatment strategy in the staged management.