Ectopia : cordis is an extremely rare congenital malformation where the heart is located partially or totally outside the thoracic cavity. The four main ectopic positions are::
adjacent to the thorax: ~60 %
abdominal: 15-30%
thoraco-abdominal: 7-18%
cervical: ~3%
Epidemiology
The estimated prevalence is 1:126,000 births. There may be a slight female preponderance ref.
Pathology
It results from the failure of migration of lateral mesoderm into the midline.
Ectopia cordis may occur as an isolated malformation or it may be associated with a larger category of ventral body wall defects that affect the thorax, abdomen or both.
Associations
Individual associations
Ventricular septal defect and tetralogy of Fallot are the most common intracardiac defects.
Omphalocele is the most common abdominal wall defect.
Syndromic associations
A well-known association is pentalogy of Cantrell 1 which comprises of:
ectopia cordis
omphalocele (typically supraumbilical)
congenital diaphragmatic hernia
sternal cleft
congenital heart disease
Radiographic features
Antenatal ultrasound
When in isolation, the heart is seen in the amniotic cavity with a thoracic wall defect. If in association with pentalogy of Cantrell it may seen within an omphalocoele 2.
Plain radiograph
Imaging clues on frontal chest radiographs include:
abnormal cardiac position and configuration
air lucency may surround the ectopic heart
sternal defect is often present
wide separation of the sternal ends of the clavicles
widening of the superior mediastinum
The lateral view may confirm the extrathoracic location of the heart.
Treatment and prognosis
The prognosis is generally poor and depends on the severity of intracardiac malformations and the presence of associated abnormalities. Most infants are stillborn or die within the first hours or days of life.
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