Abstract 2631: Hypoplastic Left Heart Syndrome and Intact or Highly Restrictive Atrial Septum: Outcome in the Current Era of Prenatal Diagnosis
Objectives: We sought to review our recent experience with hypoplastic left heart syndrome (HLHS) and intact (IAS) or highly restrictive atrial septum (HRAS) and determine factors that may impact outcome and guide appropriate intervention.
Background: Despite recent improvements in survival of patients with HLHS, those with IAS or HRAS continue to face high mortality.
Methods: Medical records and echocardiograms of patients with HLHS and IAS or HRAS born between 1997 and 2006 were reviewed. IAS was defined as those with no interatrial communication and normal pulmonary venous return at birth; HRAS was defined as those where the only source for egress of pulmonary venous return was through a restrictive patent foramen ovale of ≤2mm, or through partial anomalous pulmonary venous connections.
Results: Thirty-six pts were identified; 12 (33%) IAS and 24 (67%) HRAS. Initial intervention was a Stage 1 Norwood in 27 (75%), atrial septectomy in 4 (11%), and catheter intervention in 5 (14%). Neonatal hospital survival was 27/36 (75%) overall; 23/24 (96%) for HRAS, 4/12 (33%) for IAS. Kaplan-Meier survival at 1000 days was 25% for IAS and 75% for HRAS. Type of intervention or anatomical features such as presence of decompressing vessels, atrial septal morphology, or left atrial size did not correlate with mortality. Prenatal diagnosis was made in 23 (64%) and did not impact outcome. Of the 12 with IAS, 7 had prenatal diagnosis of which 5 died. Four pts with prenatal diagnosis of IAS had scheduled Caesarian section delivery with immediate neonatal surgery at an adjacent operating room at < 1 hour of life; there was only 1 survivor. Two underwent fetal intervention at another institution; both died after neonatal hospitalization. Significant progression of atrial septal restriction from fetal to postnatal life was identified in 6; 4 with HRAS as a fetus that progressed to IAS at birth.
Conclusion: HLHS with IAS continues to be a highly lethal condition, despite prenatal diagnosis, or the mode or timing of postnatal intervention. This suggests a developmental pathology that may not be readily amenable to postnatal care alone. Fetal intervention may offer the best hope of survival for IAS, however the most effective technique and timing of intervention are yet to be determined.