Abstract
Background: Ectopia Cordis (EC) is a rare congenital condition where the heart is partially or completely lies outside the thoracic cavity (extrathoracic), uncovered by pericardium and skin. Many works of literature reported EC is a rare congenital abnormality with an incidence of about 5-8 per 1 million live births and includes about 0.1% of congenital heart diseases. Methods: This was a male baby, weighing 2.900 kg, received with cyanosis with a defect in the anterior chest wall and heart protruding out through it. On initial physical examination, split sternum with complete thoracic EC (beating outside the thoracic cavity with a complete absence of the pericardium with the apex pointing upwards) were reported. Results: The baby’s poor general condition did not allow further radiologic studies and echocardiography could not be performed. By the time, an arrangement had been planned for him to undergo cardiothoracic referral; unfortunately, he ran a downhill course and succumbed within 36 hours of life. Conclusion: The important for submitting such findings will assist our team: pediatric surgeons, obstetricians, pediatricians and, neonatal intensivists to develop future management strategies when they are enrolled or confronted with such cases, by improving the outcome through a precise workup design to provide the optimal evaluation, diagnosis, and management roadmaps of potential cases of EC.
Highlights
Ectopia Cordis (EC) is a rare congenital condition where the heart is partially or completely lies outside the thoracic cavity, uncovered by pericardium and skin [1]
Many works of literature reported EC is a rare congenital abnormality with an incidence of about 5-8 per 1 million live births and includes about 0.1% of congenital heart diseases
And through the intrauterine fetal life, it occurs because of the failure of maturation of the midline mesodermal components of the chest and abdomen. It may occur as an isolated malformation or it may be associated with body wall defects that affect the thorax, abdomen, or both [3]
Summary
Ectopia Cordis (EC) is a rare congenital condition where the heart is partially or completely lies outside the thoracic cavity (extrathoracic), uncovered by pericardium and skin. Many works of literature reported EC is a rare congenital abnormality with an incidence of about 5-8 per 1 million live births and includes about 0.1% of congenital heart diseases. Methods: This was a male baby, weighing 2.900 kg, received with cyanosis with a defect in the anterior chest wall and heart protruding out through it. Split sternum with complete thoracic EC (beating outside the thoracic cavity with a complete absence of the pericardium with the apex pointing upwards) were reported. Results: The baby’s poor general condition did not allow further radiologic studies and echocardiography could not be performed. Conclusion: The important for submitting such findings will assist our team: pediatric surgeons, obstetricians, pediatricians and, neonatal intensivists to develop future management strategies when they are enrolled or confronted with such cases, by improving the outcome through a precise workup design to provide the optimal evaluation, diagnosis, and management roadmaps of potential cases of EC
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