Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center.
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.
Vida
Vladimiro L
VL
Department of Cardiac Surgery, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
Bacha
Emile A
EA
Larrazabal
Alesandro
A
Gauvreau
Kimberly
K
Thiagaragan
Ravi
R
Fynn-Thompson
Francis
F
Pigula
Frank A
FA
Mayer
John E
JE
del Nido
Pedro J
PJ
Tworetzky
Wayne
W
Lock
James E
JE
Marshall
Audrey C
AC
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*