Presentation
Shortness of breath, fever, and cough of 4-day duration. Examination showed a bulged pulsatile mass covered with skin.
Patient Data
A frontal chest radiograph demonstrates patchy bilateral opacities throughout bilateral lungs, representing active airspace disease, most likely an infectious process.
CT thorax with pulmonary angiogram protocol was performed to evaluate SOB and chest pain, demonstrating partial ectopia cordis with right ventricular outflow tract herniation. The heart is seen herniated through a midline defect in the sternum (sternal cleft) and lying outside the thorax, covered by skin. An atrial septal defect is also noted.
The lung window image shows diffuse bilateral ground-glass/consolidative opacities in a peripheral distribution. Considering that we are in the COVID-19 pandemic era, it was suspected.
Case Discussion
Ectopia cordis (EC) is an extremely rare congenital abnormality. It manifested as herniation of the heart outside the thorax covered with or without the skin.
The prevalence of EC is about 5.5–7.9 per million live births. Most of which died within a few days or weeks if they did not have a surgical intervention. With surgical intervention, the patient can live for a few months or years. The first case report of this entity was back in 1706.
This is the first case for a patient with EC who lives until adulthood without surgical intervention. In the end, unfortunately, the patient died because of COVID-19 pneumonia, not because of the congenital abnormality itself 1.
Informed consent has been obtained from the family.