What Is Ectopia Cordis

What Is Ectopia Cordis?

Ectopia cordis is a condition where the fetus develops without any lungs or other vital organs. Some cases are fatal, but others survive with only minor problems. There are two types of ectopia cordis: pulmonary and nonpulmonary. Pulmonary ectopia cordis usually occurs when there is no connection between the umbilical vein and the heart (abnormality). Nonpulmonary ectopia cordis happens when the umbilical vein does not connect to the heart at all (congenital abnormality).

Pulmonary ectopia cordis is a rare condition that affects less than 1% of live births. It is caused by a defect in one of the fetal lung structures called the trachea. A hole forms in it, which prevents oxygenated blood from getting into the baby’s body. Without the lungs, the baby dies within hours after birth.

Most babies born with pulmonary ectopia cordis die within 24 hours.

Nonpulmonary ectopia cordis is a much more common problem that affects up to 20% of live births. It results from a normal development of certain cells in the placenta, which then develop abnormally and block off blood flow to parts of the body such as the brain and heart. This causes various heart defects, most commonly a ventricular septal defect. Other possible problems include interatrial and interventricular septal defects, atrial septal defects, patent ductus arteriosus, tetralogy of fallot, transposition of the great arteries, aortic stenosis, truncus arteriosus, and atrioventricular septal defect.

There are two types of nonpulmonary ectopia cordis based on where the septum develops: endocardial and myocardial. Endocardial means that it forms between the endomyocardium and myocardium layers of the heart, while myocardial means it forms within the myocardium layer. Part of the septum may be covered by endomyocardium or myocardium, but not both.

Sources & references used in this article:

Ectopia cordis and other midline defects by JM Morales, SG Patel, JA Duff, RL Villareal… – The Annals of thoracic …, 2000 – Elsevier

Outcome of patients with ectopia cordis and significant intracardiac defects. by LK Hornberger, SD Colan, JE Lock, DL Wessel… – Circulation, 1996 – ncbi.nlm.nih.gov

Staged repair of ectopia cordis by ARC Dobell, HB Williams, RW Long – Journal of pediatric surgery, 1982 – Elsevier

Embryology, sternal clefts, ectopia cordis, and Cantrell’s pentalogy by SA Engum – Seminars in pediatric surgery, 2008 – Elsevier