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Presentation and outcomes of ectopia cordis
T Humpl1, P Huggan, L K Hornberger
1The Hospital for Sick Children, Toronto, Canada.
The Canadian Journal of Cardiology
|January 6, 2000
Summary
Ectopia cordis, a rare heart malformation, has a high mortality rate in infants, particularly when accompanied by structural heart disease or other congenital anomalies. Early diagnosis via prenatal ultrasound is possible, but outcomes remain poor.
Area of Science:
- Pediatric Cardiology
- Congenital Malformations
- Medical Imaging
Background:
- Ectopia cordis is a rare condition where the heart is displaced outside the chest.
- Its exact cause and pathogenesis are unknown.
- It often co-occurs with structural heart disease and noncardiac malformations.
Purpose of the Study:
- To review the clinical presentation, diagnostic methods, management strategies, and outcomes of infants diagnosed with ectopia cordis.
- Focus on cases managed at The Hospital for Sick Children, Toronto.
Main Methods:
- Retrospective review of medical records for patients with ectopia cordis.
- Analysis of echocardiograms, surgical reports, and autopsy findings.
Main Results:
- Ten patients with ectopia cordis were identified between 1978 and 1998.
- Three had normal cardiac anatomy; seven had associated cardiac anomalies.
- Six cases involved noncardiac malformations, and six were diagnosed prenatally.
- All infants in the study died, either shortly after birth, during surgery, or in the postoperative period.
Conclusions:
- Despite advances in medical care, mortality for ectopia cordis remains high.
- The presence of additional structural heart disease or other malformations significantly worsens the prognosis.