ECTOPIA CORDIS

Ectopia cordis is a rare malformation resulting in evisceration of the heart through a ventral wall defect.

• Embryology.
• Pathogenesis - Unknown. No predisposing factors or familial association has been described. It arises due to failure of the cephalic body folds to fuse.
• Classification (1) - It may present as an isolated lesion or part of the pentalogy of Cantrell.

Cervical	3%	Displacement of the heart into the neck area.
Thoracic	60%	Heart is outside thoracic cavity through a sternal defect.
Thoracoabdominal	7%	The heart is displaced outside the chest through a defect in the lower sternum with an associated Diaphragmatic or ventral abdominal wall defect (pentalogy of Cantrell).
Abdominal	30%	Heart is displaced into the abdomen through a defect in the diaphragm.

ULTRASOUND (2-9)

• The entire heart, or less commonly a portion of the heart, is eviscerated outside the thoracic cavity.
• Thoracic.
• Sternal defect, partial absence of parietal pericardium, protrusion of part or all of the heart through the defect, and omphalocele in most cases.
• Thoracoabdominal.
• Diaphragmatic defect.
• Intrinsic cardiac defects.
• Pentalogy of Cantrell.

ASSOCIATED ANOMALIES

Link to Associated Anomalies

OUTCOME

No reported cases of survival.

REFERENCES

1. Ravitch MM. Congenital deformities of the chest wall and their operative correction: Ectopia cordis. Saunders, Philadelphia 1977:11-23.
2. Wicks JD, Levine MD, Mettler FA. Intrauterine sonography of thoracic ectopia cordis. AJR 1981;137:619-622.
3. Mercer LJ, Petres RE, Smetlzer JS. Ultrasound diagnosis of of ectopia cordis. Obstet Gynecol 1983;661:523.
4. Baker ME, Rosenberg ER, Trofatter KF et.al. The in utero findings in twin pentalogy of Cantrell. J Ultrasound Med 1984;3:525-527.
5. Haynor DR, Shuman WP, Brewer Dr et.al. Imaging of fetal ectopia cordis: role of sonography and computed tomography. J Ultrasound Med 1984;3:25-28.
6. Seeds JW, Cephalo RC, Lies SC et.al. Early prenatal sonographic appearance of rare thoraco-abdominal eventration. Prenat Diagn 1994;4:437-441.
7. Abu-Yousel MM, Wray AB, Williamson RA et.al. Antenatal ultrasound diagnosis of variant of pentalogy of Cantrell. J Ultrasound Med 1987;6:535-538.
8. Klingensmith WC III, Cioffi-Ragan DT, Harvey DE. Diagnosis of ectopia cordis in the second trimester. J Clin Ultrasound 1988;16:204-206.
9. Liang R -I, Huang S -E, Chang F -M. Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography. Ultrasound Obstet Gynecol 1997;10:137-139.