ectopia cordis

Ectopia

cordis • Ectopia cordis - Ganzer Fall bei Radiopaedia

Tatco V, Ectopia cordis. Case study, Radiopaedia.org (Accessed on 02 Oct 2023) https://doi.org/10.53347/rID-41893 • CC by-nc-sa 3.0 (modified)

Ectopia

cordis • Ectopia cordis - Ganzer Fall bei Radiopaedia

Andrews M, Ectopia cordis. Case study, Radiopaedia.org (Accessed on 02 Oct 2023) https://doi.org/10.53347/rID-60365 • CC by-nc-sa 3.0 (modified)

Ectopia

cordis • Ectopia cordis with COVID-19 pneumonia - Ganzer Fall bei Radiopaedia

Al-Briek A, Ectopia cordis with COVID-19 pneumonia. Case study, Radiopaedia.org (Accessed on 02 Oct 2023) https://doi.org/10.53347/rID-97669 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

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%
thoracoabdominal: 7-18%
cervical: ~3%

Epidemiology

The incidence is 5.5 to 7.9 per 1,000,000 live births .

Pathology

It results from the failure of migration of lateral mesoderm into the midline. Some forms may be the outcome of amniotic band syndrome .

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 which comprises:

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 omphalocele .

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.

CT/MRI

Cross-sectional modalities allow for the best visualization of ectopia cordis, and is required for any surgical planning.

Treatment and prognosis

Although surgical correction may be attempted , 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.