A population-based study of extra-cardiac anomalies in children with congenital cardiac malformations

Area of Science:

• Pediatric Cardiology
• Congenital Malformations
• Clinical Outcomes

Background:

• Congenital cardiac malformations are a significant concern in pediatric health.
• Extra-cardiac anomalies can influence the prognosis of congenital heart disease.
• Understanding these associations is crucial for comprehensive patient care.

Purpose of the Study:

• To determine the prevalence of extra-cardiac anomalies in children with congenital cardiac malformations.
• To compare the survival impact of these anomalies versus isolated cardiac lesions.
• To analyze survival trends over time in relation to extra-cardiac anomalies.

Main Methods:

• Retrospective analysis of 3527 children with congenital cardiac anomalies.
• Data collected from a tertiary Pediatric Cardiology center in Norway (1990-1999).
• Comparison of survival rates between children with and without extra-cardiac anomalies, including Down syndrome.

Main Results:

• One-fifth of children with congenital heart defects had extra-cardiac anomalies.
• Down syndrome accounted for nearly one-third of these associated anomalies.
• Survival improved from 1995-1999 vs. 1990-1994 for most groups, except those with non-Down syndrome extra-cardiac anomalies.

Conclusions:

• Extra-cardiac anomalies, particularly Down syndrome, significantly impact survival in pediatric congenital heart disease.
• While survival has improved overall, a specific subgroup (non-Down syndrome extra-cardiac anomalies) shows no improvement.
• This heterogeneous group requires further investigation to understand variable survival patterns.