Ectopia cordis

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Ectopia cordis
Synonyms
Pronounce
Specialty	Cardiology, Pediatric surgery
Symptoms	Heart located outside the thoracic cavity
Complications	Infection, heart failure, death
Onset	Congenital
Duration	Lifelong
Types	Thoracic, Cervical, Thoracoabdominal, Abdominal
Causes	Developmental defect during embryogenesis
Risks
Diagnosis	Prenatal ultrasound, Echocardiogram, MRI
Differential diagnosis
Prevention
Treatment	Surgical repair
Medication
Prognosis	Poor, high mortality rate
Frequency	Rare
Deaths

Congenital malformation where the heart is located outside the thoracic cavity

Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside of the thoracic cavity. This condition is often associated with other congenital disorders and has a high mortality rate due to the exposure of the heart to the external environment and the associated complications.

Classification

Ectopia cordis can be classified based on the location of the heart:

• Cervical ectopia cordis: The heart is located in the neck region.
• Thoracic ectopia cordis: The heart is located in the chest but outside the rib cage.
• Thoracoabdominal ectopia cordis: The heart is located between the thorax and abdomen.
• Abdominal ectopia cordis: The heart is located in the abdominal region.

Pathophysiology

The exact cause of ectopia cordis is not well understood, but it is believed to result from a failure in the development of the ventral body wall during embryogenesis. This failure leads to a defect in the sternum and pericardium, allowing the heart to develop outside the thoracic cavity.

Associated Anomalies

Ectopia cordis is often associated with other congenital anomalies, including:

• Omphalocele
• Pentalogy of Cantrell
• Congenital heart defects
• Diaphragmatic hernia

Diagnosis

Ectopia cordis can be diagnosed prenatally using ultrasound imaging. The condition is often detected during routine prenatal scans, where the abnormal position of the heart can be visualized.

Management

Management of ectopia cordis is challenging and typically involves surgical intervention. The goals of surgery are to protect the heart, correct associated anomalies, and relocate the heart into the thoracic cavity if possible. The timing and approach to surgery depend on the severity of the condition and the presence of other anomalies.

Prognosis

The prognosis for infants with ectopia cordis is generally poor, with a high mortality rate. Survival depends on the extent of the defect, the presence of associated anomalies, and the success of surgical interventions.

See also

• Congenital heart defect
• Pentalogy of Cantrell
• Omphalocele