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Ectopia Cordis
Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Anomaly in which fetal heart lies completely or partially outside thoracic cavity

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Zeidler S et al: Early prenatal disruption; a foetus with features of severe limb body wall sequence, body stalk anomaly and amniotic bands. Genet Couns. 25(3):315-20, 2014
                                    2. Pepper MA et al: Thoracoabdominal wall defect with complete ectopia cordis and gastroschisis: a case report and review of the literature. Pediatr Dev Pathol. 16(5):348-52, 2013
                                    3. Sepulveda W et al: Ectopia cordis in a first-trimester sonographic screening program for aneuploidy. J Ultrasound Med. 32(5):865-71, 2013
                                    4. Sadłecki P et al: Prenatally evolving ectopia cordis with successful surgical treatment. Fetal Diagn Ther. 30(1):70-2, 2011
                                    5. Balakumar K et al: Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation. Ann Pediatr Cardiol. 3(2):166-8, 2010
                                    6. Barbee K et al: First-trimester prenatal sonographic diagnosis of ectopia cordis in a twin gestation. J Clin Ultrasound. 37(9):539-40, 2009
                                    7. Gao Z et al: Prognosis of pentalogy of Cantrell depends mainly on the severity of the intracardiac anomalies and associated malformations. Eur J Pediatr. 168(11):1413-4, 2009
                                    8. Engum SA: Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg. 17(3):154-60, 2008
                                    9. Moniotte S et al: Prenatal diagnosis of thoracic ectopia cordis by real-time fetal cardiac magnetic resonance imaging and by echocardiography. Congenit Heart Dis. 3(2):128-31, 2008
                                    10. Yildirim G et al: Prenatal diagnosis of ectopia cordis. Taiwan J Obstet Gynecol. 47(3):346-7, 2008
                                    11. Gonçalves FD et al: Thoracic ectopia cordis with anatomically normal heart. Rev Bras Cir Cardiovasc. 22(2):245-7, 2007
                                    12. Bianca S et al: Prenatal 2-dimensional and 3-dimensional ultrasonography diagnosis and autoptic findings of isolated ectopia cordis. Cardiology. 105(1):37-40, 2006
                                    13. Twomey EL et al: Prenatal ultrasonography and neonatal imaging of complete cleft sternum: a case report. Ultrasound Obstet Gynecol. 25(6):599-601, 2005
                                    14. Ley EJ et al: Successful repair of ectopia cordis using alloplastic materials: 10-year follow-up. Plast Reconstr Surg. 114(6):1519-22, 2004
                                    15. Humpl T et al: Presentation and outcomes of ectopia cordis. Can J Cardiol. 15(12):1353-7, 1999
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Definitions

                                                • Anomaly in which fetal heart lies completely or partially outside thoracic cavity

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Zeidler S et al: Early prenatal disruption; a foetus with features of severe limb body wall sequence, body stalk anomaly and amniotic bands. Genet Couns. 25(3):315-20, 2014
                                                                      2. Pepper MA et al: Thoracoabdominal wall defect with complete ectopia cordis and gastroschisis: a case report and review of the literature. Pediatr Dev Pathol. 16(5):348-52, 2013
                                                                      3. Sepulveda W et al: Ectopia cordis in a first-trimester sonographic screening program for aneuploidy. J Ultrasound Med. 32(5):865-71, 2013
                                                                      4. Sadłecki P et al: Prenatally evolving ectopia cordis with successful surgical treatment. Fetal Diagn Ther. 30(1):70-2, 2011
                                                                      5. Balakumar K et al: Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation. Ann Pediatr Cardiol. 3(2):166-8, 2010
                                                                      6. Barbee K et al: First-trimester prenatal sonographic diagnosis of ectopia cordis in a twin gestation. J Clin Ultrasound. 37(9):539-40, 2009
                                                                      7. Gao Z et al: Prognosis of pentalogy of Cantrell depends mainly on the severity of the intracardiac anomalies and associated malformations. Eur J Pediatr. 168(11):1413-4, 2009
                                                                      8. Engum SA: Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg. 17(3):154-60, 2008
                                                                      9. Moniotte S et al: Prenatal diagnosis of thoracic ectopia cordis by real-time fetal cardiac magnetic resonance imaging and by echocardiography. Congenit Heart Dis. 3(2):128-31, 2008
                                                                      10. Yildirim G et al: Prenatal diagnosis of ectopia cordis. Taiwan J Obstet Gynecol. 47(3):346-7, 2008
                                                                      11. Gonçalves FD et al: Thoracic ectopia cordis with anatomically normal heart. Rev Bras Cir Cardiovasc. 22(2):245-7, 2007
                                                                      12. Bianca S et al: Prenatal 2-dimensional and 3-dimensional ultrasonography diagnosis and autoptic findings of isolated ectopia cordis. Cardiology. 105(1):37-40, 2006
                                                                      13. Twomey EL et al: Prenatal ultrasonography and neonatal imaging of complete cleft sternum: a case report. Ultrasound Obstet Gynecol. 25(6):599-601, 2005
                                                                      14. Ley EJ et al: Successful repair of ectopia cordis using alloplastic materials: 10-year follow-up. Plast Reconstr Surg. 114(6):1519-22, 2004
                                                                      15. Humpl T et al: Presentation and outcomes of ectopia cordis. Can J Cardiol. 15(12):1353-7, 1999