BACKGROUND: Ectopia cordis, defined as complete or partial displacement of the heart outside the thoracic cavity, is rare. The cause and pathogenesis are unknown. The anatomy of the heart may be normal, but the presence of structural heart disease as well as noncardiac malformations is common. Patients present incidentally after birth or, more recently, the condition has been diagnosed by prenatal echocardiography. OBJECTIVE: To review the presentation, diagnostic imaging, management and outcomes of infants with ectopia cordis at The Hospital for Sick Children, Toronto, Ontario. METHODS: The medical records of the database of the Division of Cardiology, The Hospital for Sick Children, were searched; echocardiograms, and surgical and autopsy reports were further reviewed. RESULTS: Between 1978 and 1998, 10 patients with ectopia cordis presented to the authors' centre. Normal cardiac anatomy was noted in three, and mild to complex structural cardiac anomalies were noted in the remainder. Associated noncardiac malformations were found in six cases. Six were diagnosed by prenatal ultrasound between 19 and 37 weeks of gestation. Pregnancies were electively terminated or the infants died shortly after birth. Four other neonates with ectopia cordis were referred to the authors' centre. All of these infants subsequently died either at the time of surgery for cardiac and noncardiac anomalies or in the immediate postoperative period. CONCLUSIONS: Despite modern treatment options, the mortality remains high for patients with ectopia cordis, especially in the presence of structural heart disease or other malformations.
BACKGROUND:Ectopia cordis, defined as complete or partial displacement of the heart outside the thoracic cavity, is rare. The cause and pathogenesis are unknown. The anatomy of the heart may be normal, but the presence of structural heart disease as well as noncardiac malformations is common. Patients present incidentally after birth or, more recently, the condition has been diagnosed by prenatal echocardiography. OBJECTIVE: To review the presentation, diagnostic imaging, management and outcomes of infants with ectopia cordis at The Hospital for Sick Children, Toronto, Ontario. METHODS: The medical records of the database of the Division of Cardiology, The Hospital for Sick Children, were searched; echocardiograms, and surgical and autopsy reports were further reviewed. RESULTS: Between 1978 and 1998, 10 patients with ectopia cordis presented to the authors' centre. Normal cardiac anatomy was noted in three, and mild to complex structural cardiac anomalies were noted in the remainder. Associated noncardiac malformations were found in six cases. Six were diagnosed by prenatal ultrasound between 19 and 37 weeks of gestation. Pregnancies were electively terminated or the infants died shortly after birth. Four other neonates with ectopia cordis were referred to the authors' centre. All of these infants subsequently died either at the time of surgery for cardiac and noncardiac anomalies or in the immediate postoperative period. CONCLUSIONS: Despite modern treatment options, the mortality remains high for patients with ectopia cordis, especially in the presence of structural heart disease or other malformations.