- What causes this malformation
- What happens when the heart is out of the chest
- What are the treatment options
- How to confirm the diagnosis
Ectopia cordis, also known as cardiac ectopia, is a very rare malformation in which the baby's heart is located outside the breast, under the skin. In this malformation, the heart may be located completely outside the chest or only partially outside the chest.
In most cases, there are other associated malformations and, therefore, the average life expectancy is few hours, and most babies end up not surviving after the first day of life. Ectopia cordis can be identified in the first trimester of pregnancy, through ultrasound examination, but there are also rarer cases in which the malformation is only observable after birth.
In addition to defects in the heart, this disease is also associated with defects in the structure of the chest, abdomen and other organs, such as the intestine and lungs. This problem must be treated with surgery to put the heart back in place, but the risk of death is high.
What causes this malformation
The specific cause of ectopia cordis is not yet known, however, it is possible that the malformation arises due to an incorrect development of the sternum bone, which ends up being absent and allowing the heart to pass out of the breast, even during pregnancy.
What happens when the heart is out of the chest
When the baby is born with the heart out of the breast, it usually also has other health complications such as:
- Defects in the functioning of the heart; Defects in the diaphragm, leading to difficulty in breathing; Intestine out of place.
The baby with ectopia cordis has a greater chance of survival when the problem is only the poor location of the heart, without other associated complications.
What are the treatment options
Treatment is only possible through surgery to replace the heart and reconstruct defects in the chest or other organs that have also been affected. Surgery is usually done in the first days of life, but it will depend on the severity of the disease and the baby's health.
However, cordis ecotopia is a serious problem and in most cases leads to death in the first days of life, even when surgery is performed. Parents of children with this disease can have genetic tests to assess the chances of recurrence of the problem or other genetic defects in the next pregnancy.
In cases where the baby manages to survive, it is usually necessary to resort to several surgeries throughout his life, as well as to maintain regular medical care, to ensure that there are no life-threatening complications.
How to confirm the diagnosis
The diagnosis can be made from the 14th week of pregnancy, through conventional and morphological ultrasound exams. After the diagnosis of the problem, other ultrasound exams should be done frequently to monitor the development of the fetus and the worsening or not of the disease, so that the delivery through cesarean section is scheduled.
