[Favourable results with surgical treatment in 43 children with hypoplastic left-heart syndrome or similar disorders, 1999-2005].
OBJECTIVE
To describe the results of surgical treatment of hypoplastic left-heart syndrome (HLHS) and HLHS-like disorders in the Amsterdam-Leiden Centre for Congenital Heart Disease, the Netherlands.


METHODS
Retrospective, descriptive.


METHODS
Data were collected on 43 neonates with HLHS or similar disorders who underwent surgical treatment between December 1999 and December 2005. HLHS was present in 37 patients and 6 had disorders similar to HLHS (unbalanced atrioventricular septal defect, truncus arteriosus with hypoplastic left ventricle, double inlet left ventricle). Surgery was performed in 3 steps: Norwood operation shortly after birth (n = 43), bidirectional cavopulmonary anastomosis a few months later (n = 30) and total cavopulmonary connection at the age of 2-3 years (n = 10). During the Norwood operation, the first 21 patients received a modified Blalock shunt (between the right brachiocephalic artery and pulmonary artery), whereas the following 22 patients received a Sano shunt (between the right ventricle and pulmonary artery).


RESULTS
Of the 43 patients, 11 died: 7 within 30 days of the first operation, 2 between the first and second operation, and 2 between the second and third operation. Actuarial survival for the entire group is 74% (32/43). The mortality rate was lower with the Sano shunt (9%; 2/22) than with the modified Blalock shunt (43%; 9/21). Catheter interventions were necessary in 10 patients: 6 had balloon dilatation of the distal aortic arch and 4 had balloon dilatation/stent placement for narrowed pulmonary arteries. With a median follow-up of 22 months (range: 1-75), 2 patients had marked neurological side effects. All 32 surviving patients were in good clinical condition.
Hazekamp
M G
MG
Leids Universitair Medisch Centrum, Postbus 9600, 2300 RC Leiden. m.g.hazekamp@lumc.nl
Rijlaarsdam
M E B
ME
Schoof
P H
PH
Wald
A A M
AA
Blom
N A
NA
Ottenkamp
J
J
D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012086:Reoperation; D012189:Retrospective Studies; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome