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Complete thoracic ectopia cordis.

N Alphonso1, P S Venugopal, R Deshpande

  • 1Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Trust, St Thomas' Street, London SE1 9RT, UK. nelson.a@bigfoot.com

European Journal of Cardio-Thoracic Surgery : Official Journal of the European Association for Cardio-Thoracic Surgery
|March 5, 2003
PubMed
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Thoracic ectopia cordis, a rare congenital defect, presents significant surgical challenges. This case highlights the difficulties in achieving long-term survival despite early intervention for this complex heart defect.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Pediatric Surgery

Background:

  • Thoracic ectopia cordis is a rare congenital anomaly where the heart is abnormally positioned outside the chest cavity.
  • Survival rates after surgical correction are historically low, underscoring the condition's severity.
  • This case involves a rare combination of complete thoracic ectopia cordis and double outlet right ventricle.

Observation:

  • Antenatal diagnosis of complete thoracic ectopia cordis with double outlet right ventricle was achieved.
  • Surgical repair was performed shortly after birth.
  • The infant demonstrated initial stability, including successful extubation on postoperative day five.

Findings:

  • Despite initial stability, the infant experienced an unexplained respiratory arrest 48 hours after extubation, leading to mortality.

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  • This outcome underscores the critical challenges and poor prognosis associated with surgical correction of severe thoracic ectopia cordis.
  • A review of current surgical strategies for this condition is also presented.

    • Implications:

    • The case emphasizes the need for improved diagnostic and therapeutic strategies for thoracic ectopia cordis.
    • Further research into the underlying mechanisms and long-term outcomes of surgical interventions is warranted.
    • This report contributes to the limited literature on surgical management and survival in complex congenital heart defects like ectopia cordis.