Surgical treatment of hypoplastic left heart syndrome: limited success
Plantinga, R.F.; Frohn-Mulder, I.M.; Takkenberg, H.J.; Bennink, G.B.; Meijboom, E.J.; Bogers, A.J.
Nederlands Tijdschrift Voor Geneeskunde 145(2): 85-90
2001
ISSN/ISBN: 0028-2162 PMID: 11225263 Document Number: 529524
To inventory the treatment of hypoplastic left heart syndrome (HLHS) in the Netherlands and its results. Retrospective. Data were collected from all patients (n = 117) diagnosed with HLHS in the Wilhelmina Children's Hospital-University Medical Center Utrecht and the University Hospital Rotterdam-Sophia Children's Hospital and born in the period 1 March 1988-31 May 2000. Type and time of intervention, and mortality were recorded and cumulative survival was analysed by Kaplan-Meier analysis. Cumulative survival was compared between early and late series and between the two hospitals. The study group comprised 68 boys and 49 girls, all neonates. At the time of the investigation, the mean duration of follow-up was 185 days (range: 0-3855). Fifty-eight children had received no treatment; all of these had died. Fifty-nine children were scheduled for the Norwood procedure; six of them died before operation. The 53 patients who underwent the first stage of the Norwood procedure had 1-month, 1-year, 2-year, and 5-year survival chances of 55%, 30%, 27%, and 24% respectively. Survival chances between the two time periods and the two hospitals showed no significant differences. The Norwood procedure was performed in almost half of the children with HLHS. It is only moderately successful; however, it seems the only realistic choice in the management.