title: Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center.
abstract: BACKGROUND
  The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors.
  
  
  METHODS
  We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006. Chart review enabled analysis of patient and procedural variables.
  
  
  RESULTS
  All 32 patients underwent left atrial decompression in utero or postnatally before surgery. Fourteen patients (44%) underwent fetal intervention, either atrial septoplasty (n = 9) or aortic valvuloplasty (n = 5). Twenty-nine of the 32 patients had postnatal left atrial hypertension and underwent transcatheter atrial septoplasty as neonates before surgery; 3 did not require postnatal atrial septoplasty after successful fetal atrial septoplasty. After stage I, hospital survival was 69% (22 of 32). Need for shunt revision (p = 0.02) and for extracorporeal membrane oxygenation use (p < 0.001) were associated with hospital mortality. Survival at 6 months was 69% for patients who had fetal intervention, and 38% for those who were treated only postnatally (p = 0.2).
  
  
  CONCLUSIONS
  Surgical outcome for patients with HLHS and I/HRAS continues to improve. Prenatal decompression of the left atrium may be associated with greater hospital survival. Proposed effects of fetal intervention on lung pathology and longer-term survival are subjects for future study in this unique group of patients.
authors.affiliation: Department of Cardiac Surgery, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
authors.forename: Vladimiro L
authors.initials: VL
authors.lastname: Vida
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.forename: Alesandro
authors.initials: A
authors.lastname: Larrazabal
authors.forename: Kimberly
authors.initials: K
authors.lastname: Gauvreau
authors.forename: Ravi
authors.initials: R
authors.lastname: Thiagaragan
authors.forename: Francis
authors.initials: F
authors.lastname: Fynn-Thompson
authors.forename: Frank A
authors.initials: FA
authors.lastname: Pigula
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: Wayne
authors.initials: W
authors.lastname: Tworetzky
authors.forename: James E
authors.initials: JE
authors.lastname: Lock
authors.forename: Audrey C
authors.initials: AC
authors.lastname: Marshall
mesh_terms: D006328:Cardiac Catheterization / Q000379:methods*; D006348:Cardiac Surgical Procedures / Q000379:methods*; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005318:Fetal Heart; D006325:Heart Atria / Q000033:anatomy & histology* / Q000196:embryology; D006346:Heart Septum / Q000033:anatomy & histology*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000196:embryology / Q000601:surgery*; D007231:Infant, Newborn; D008946:Mitral Valve Stenosis / Q000601:surgery; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D014188:Transposition of Great Vessels / Q000175:diagnosis*