A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction.
OBJECTIVE
We compare the hemodynamics and perioperative course of shunt type in hypoplastic left heart syndrome at the time of stage 2 reconstruction and longer-term survival.


METHODS
We retrospectively reviewed the echocardiograms, catheterizations, and hospital records of all patients who had a stage 1 reconstruction between January 2002 and May 2005 and performed a cross-sectional analysis of hospital survivors.


RESULTS
One hundred seventy-six patients with hypoplastic left heart syndrome and variants underwent a stage 1 reconstruction with either a right ventricle-pulmonary artery conduit (n = 62) or a modified Blalock-Taussig shunt (n = 114). The median duration of follow-up is 29.1 months (range, 0-57 months). By means of Kaplan-Meier analysis, there is no difference in survival at 3 years (right ventricle-pulmonary artery conduit: 73% [95% confidence limit, 59%-83%] vs modified Blalock-Taussig shunt: 69% [95% confidence limit, 59%-77%]; P = .6). One hundred twenty-four patients have undergone stage 2 reconstruction (78 modified Blalock-Taussig shunts and 46 right ventricle-pulmonary artery conduits). At the time of the stage 2 reconstruction, patients with right ventricle-pulmonary artery conduits were younger (153 days [range, 108-340 days]; modified Blalock-Taussig shunt, 176 days [range, 80-318 days]; P = .03), had lower systemic oxygen saturation (73% [range, 58%-85%] vs 77% [range, 57%-89%], P < .01), and had higher preoperative hemoglobin levels (15.8 g/dL [range, 13-21 g/dL] vs 14.8 g/dL [range, 12-19 g/dL], P < .01) compared with those of the modified Blalock-Taussig shunt group. By means of echocardiographic evaluation, there was a higher incidence of qualitative ventricular dysfunction in patients with right ventricle-pulmonary artery conduits (14/46 [31%] vs 9/73 [12%], P = .02). However, no difference was observed in common atrial pressure or the arteriovenous oxygen difference.


CONCLUSIONS
Interim analyses suggest no advantage of one shunt type over another. This report raises concern of late ventricular dysfunction and outcome in patients with a right ventricle-pulmonary artery conduit.
Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pa 19119, USA. ballweg@email.chop.edu
Jean A
JA
Ballweg
Troy E
TE
Dominguez
Chitra
C
Ravishankar
Jacqueline
J
Kreutzer
Bradley S
BS
Marino
Geoffrey L
GL
Bird
Peter J
PJ
Gruber
Gil
G
Wernovsky
J William
JW
Gaynor
Susan C
SC
Nicolson
Thomas L
TL
Spray
Sarah
S
Tabbutt
D006328:Cardiac Catheterization; D016009:Chi-Square Distribution; D002648:Child; D003430:Cross-Sectional Studies; D004452:Echocardiography; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000601:surgery*; D008297:Male; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D016896:Treatment Outcome