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Decreased prevalence of hypoplastic left heart syndrome in Malta
1Paediatric Department, St. Luke's Hospital, Guardamangia, Malta.
Insights
Hypoplastic left heart syndrome (HLHS), a severe congenital heart defect, is less common in Malta than expected. This study investigates the low birth prevalence of HLHS in Malta, suggesting unique genetic or environmental factors may be involved.
Area of Science:
- Cardiology
- Pediatrics
- Genetics
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect (CHD).
- Historically considered fatal, HLHS is now treatable with neonatal heart transplantation or staged palliative surgery.
- Understanding HLHS prevalence is crucial for public health and resource allocation.
Purpose of the Study:
- To determine the birth prevalence of HLHS in Malta.
- To compare Malta's HLHS prevalence with international data.
- To investigate potential reasons for observed differences in HLHS prevalence.
Main Methods:
- Retrospective analysis of HLHS cases in Malta from 1977-1994.
- Calculation of birth prevalence for classical and HLHS-like cases.
- Statistical comparison with historical and European registry data.
Main Results:
- The birth prevalence of classical HLHS in Malta was 0.04 per 1000 live births.
- Combined HLHS and HLHS-like cases had a prevalence of 0.06 per 1000 live births.
- Malta's HLHS prevalence was significantly lower than historical and European data.
Conclusions:
- Malta exhibits a lower-than-expected birth prevalence of HLHS.
- The spectrum of CHD in Malta shows a predominance of right ventricular outflow obstruction.
- Genetic and/or environmental factors may contribute to the low HLHS rate in Malta.
Abstract:
Hypoplastic left heart syndrome (HLHS) is the most severe form of congenital heart disease (CHD), which until recently was considered to be fatal. However, surgical intervention is now possible using neonatal heart transplantation or three-stage palliative surgery (Norwood's staged operations). In Malta, during the period 1977-1994, there were four cases of classic HLHS, with an additional three cases of HLHS-like cases. The birth prevalence of classical HLHS was 0.04 per 1000 live births, whereas that of combined HLHS-like cases was 0.06 per 1000 live births for the same period. One neonate with HLHS is expected every 3 or 4 years. Despite a high rate of ascertainment of CHD, the birth prevalence of HLHS in Malta was significantly lower than that quoted in historical studies (p < 0.006) and in the European Congenital Anomalies and Twins Registry (p < 0.002). Malta has the expected birth prevalence of CHD, but the spectrum of CHD exhibits a predominance of lesions causing right ventricular outflow obstruction and a deficit of lesions causing left ventricular outflow tract obstruction. CHD is caused by a genetic-environmental interaction. The low rate of HLHS in Malta appears to be a true divergence from the usual observed spectrum of CHD and may be caused by genetic and/or environmental factors.