Ectopia Cordis is a birth defect in which the heart is abnormally located. The malfunction happens when the sternum forms during the gestation period and the heart begins to grow in the wrong place.
In the most common form, the heart protrudes outside the chest through a split sternum. The ectopic heart is not protected by the skin or sternum. Other organs may also have formed outside the skin. Less often the heart may be situated in the abdominal cavity or neck.
Often other birth defects are also present. This condition is usually fatal in the first days of life. In some cases surgical treatment is possible.
Often the heart is not formed properly and many other heart defects are associated with this condition including:
- Tetralogy of Fallot
- Pulmonary Atresia
- Atrial and Ventricular Septal Defects
- Double Outlet Right Ventricle
Other non cardiac malformations may be present such as cleft palates and spine malformations that can cause kyphosis.
Occurrence is .79 in every 10,000 births. Ectopia cordis is a very rare congenital heart malformation.
No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome and Trisomy 18; however so far there is no evidence that it is a genetically transmissible disease.
Depending on the position of the heart from birth ectopia cordis can be classified into four categories:
- Cervical – in the neck
- Thoracic – where the heart would lie within the thoracic cavity.
- Thoracoabdominal – where the heart would lie somewhere between the thoracic and abdominal cavities.
- Abdominal – where the heart would lie in the abdominal cavity.
Most cases of ectopia cordis are identified through routine prenatal ultrasound. When a case is suspected, the mother will be referred to a pediatric or fetal echocardiologist who will confirm the diagnosis. To give the child the best possible chance at survival, the baby will be born at a Level 3 hospital, where experienced pediatric cardiologists and surgeons are on hand to immediately address the problems. Birth may be vaginal or Caesarian, as indicated by the high-risk obstetrician.
Most cases result in stillbirth or death shortly after birth. Some cases of ectopia cordis can be treated surgically but in general involve lengthy and very complicated pediatric cardiothoracic surgery. Christopher Wall (19 August 1975) holds the Guinness World Record for the oldest living person with condition.
When parents choose to wait and see if their child can be treated, success of treatment depends on the severity of other heart defects, as well as other related problems. In most cases, cardiothoracic surgeons attempt repair of the heart defects before covering the heart and organs with prosthetic skin. A few cases of ectopia cordis have been treated successfully, but this defect still poses the biggest challenge and rate of failure for pediatric cardiothoracic surgeons.
Source: Wikipedia and WiseGeek
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