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Updated: Sep 26, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 17, 2014
[Surgical treatment of hypoplastic left heart syndrome: limited success]
R F Plantinga1, I M Frohn-Mulder, H J Takkenberg
1Universitair Medisch Centrum, Wilhelmina Kinderziekenhuis, Kinderhartcentrum, Postbus 85.090, 3508 AB Utrecht.
Insights
The Norwood procedure offers moderate success for hypoplastic left heart syndrome (HLHS) treatment in the Netherlands. Despite its limitations, it remains the primary surgical option for affected neonates.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Context:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect.
- Treatment strategies for HLHS have evolved over time.
- Netherlands national data on HLHS treatment is limited.
Purpose:
- To document the treatment approaches and outcomes for HLHS in the Netherlands.
- To evaluate the effectiveness of the Norwood procedure in a national cohort.
- To compare survival rates between different treatment periods and institutions.
Summary:
- A retrospective study analyzed 117 neonates with HLHS born between 1988 and 2000 in two Dutch centers.
- Fifty-nine neonates underwent the Norwood procedure, with 1-month, 1-year, 2-year, and 5-year survival rates of 55%, 30%, 27%, and 24%, respectively.
- No significant differences in survival were observed between early and late treatment series or between the two hospitals.
Impact:
- The Norwood procedure is the main surgical intervention for HLHS in the Netherlands.
- Current survival rates highlight the need for continued research and improved therapeutic strategies.
- This study provides valuable data for understanding HLHS management and outcomes in a specific national context.
Objective:
To inventory the treatment of hypoplastic left heart syndrome (HLHS) in the Netherlands and its results.
Design:
Retrospective.
Method:
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.
Results:
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.
Conclusion:
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.
