Abstract
Introduction: ectopia cordis is a rare congenital malformation, with an estimated incidence of 5 to 8 per million live births. It is defined as a malformation in which the heart is located in an extra-thoracic position. Ectopia cordis may occur as an isolated malformation or associated with other anomalies such as omphalocele, congenital heart disease or integrating Cantrell syndrome. The size and location of the defect influence the prognosis. Description: we report a case of a 24-year-old nulliparous woman, with no relevant family or personal history, in which the prenatal fetal ultrasound, performed at 21 weeks of gestation, revealed adefect of the anterior chest wall with exteriorization of the heart. Discussion: fetal echocardiography revealed a severe congenital heart disease. The parents decided to continue the pregnancy, after being duly informed by a multidisciplinary team. Delivery occurred at 37 weeks of gestation but the female newborn died one hour afterwards. Pathological examination confirmed the sonographic findings.
Highlights
Ectopia cordis is a rare congenital condition that is defined by the abnormal position of the heart outside the thorax associated with defects in the parietal pericardium, diaphragm, sternum, and, in most cases, cardiac malformations
Its association with a heart located outside the chest wall it is known as ectopia cordis
The thoracoabdominal type is regarded as a distinct syndrome known as Cantrells pentology, which includes five associated anomalies: distal sternum defect; midline supraumbilical abdominal wall defect; ventral diaphragmatic hernia; defect of the apical pericardium with free communication into the peritoneal cavity; and congenital intracardiac defects.[5]
Summary
Ectopia cordis is a rare congenital condition that is defined by the abnormal position of the heart outside the thorax associated with defects in the parietal pericardium, diaphragm, sternum, and, in most cases, cardiac malformations. Description: we report a case of a 24-year-old nulliparous woman, with no relevant family or personal history, in which the prenatal fetal ultrasound, performed at 21 weeks of gestation, revealed a defect of the anterior chest wall with exteriorization of the heart. Key words Ectopia cordis, Heart defects congenital, Prenatal diagnosis, Ultrasonography
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