Thorakoabdominale Ectopia cordis (Cantrell-Pentalogie): Pränatale Diagnostik und neonatologischer Verlauf

Abstract

A 17-year-old primigravida was referred at 31 + 6 weeks' gestation because of a supraumbilical fetal midline defect, thoracoabdominal ectopia cordis, and sternal aplasia. Fetal echocardiography showed a double-outlet right ventricle with pulmonary artery stenosis and a ventricular septal defect. Karyotyping was declined. After premature rupture of the membranes at 36+5 weeks'gestation a 3000-g male infant was delivered by cesarean section. The Apgar scores were 3/3 and the arterial cord blood pH was 7.26. Tissue coverage of the supraumbilical defect and the omphalocele was performed. Hemodynamic instability precluded primary replacement of the heart into the thorax. To relieve pressure a corset was fashioned for the thorax. The double-outlet right ventricle was corrected at 5 months. At 17 months the child is alive and stable. Replacement of the extrathoracic heart is planned at a later date.