ectopia cordis

both atria and ventricles lie outside the chest cavity and float freely in the amniotic cavity. the dimensions of the thorax are reduced . congenital heart disease can be associated. tetralogy of fallot is the most common associated lesion as was present here.

description

this occurs when the heart in its pericardial sac lies partially or completely outside the chest cavity. it is due to partial or complete absence of the sternum. there may be associated congenital heart malformation. there may or may not be skin covering the defect. there may be an associated omphalocoele. it may lead to spontaneous intrauterine death; if the child survives to delivery, the outcome will depend on the extent of cardiac protrusion and any associated congenital heart disease. even in the absence of chd, if there is no skin cover and the heart lies completely outside the chest cavity, it may be difficult to reconstruct a ‘cage’ around the heart to protect it in an acceptably aesthetic manner. there are few cases in the literature of successful repair.

diagnosis

part or all of the heart protrudes into the amniotic cavity from the anterior chest wall. if there is no skin cover, the heart appears to float freely in the amniotic fluid. the connections of the heart should be examined carefully for malformation. the tetralogy of fallot is the most commonly described associated lesion. the anterior abdominal wall must be examined for evidence of an associated gut protrusion.

differential diagnosis

there is no alternative diagnosis for a freely floating heart outside the chest cavity; however, a small cardiac protrusion may be difficult to differentiate from a ventricular aneurysm. in the latter, there is no sternal deficiency although this can be difficult to ascertain prenatally.

sonographic features

protrusion of the heart into the amniotic fluid, either partially or wholly.

may be any form of chd associated.

may be coexistent anterior diaphragmatic defect and/or protrusion of intestines.

associated syndromes

amniotic band /amniotic rupture sequence
diaphragmatic hernia
limb-body wall complex (body stalk anomaly)
pentalogy of cantrell
trisomy 13
trisomy 18
turner

references

anderson rh, mccartney fj, shinebourne ea, tynan m (eds) in: paediatric cardiology churchill livingstone: edinburgh, p1057-1073
allan ld, sharland gk, milburn a, lockhart sm, groves amm, anderson rh, cook ac, fagg nlk prospective diagnosis of 1,006 consecutive cases of congenital heart disease in the fetus j am coll cardiol 23:1452-8
allan ld, sharland g, cook a in: color atlas of fetal cardiology mosby-wolfe: london, p141-149
van praagh r, weinberg pm, smith sd, foran rb, van praagh s in: heart disease in infants, children and adolescents adams fh, emmanouilides gc, riemenschneider ta (eds) williams and wilkins: baltimore, p570-577
todros t, presbitero p, montemurro d, levis f prenatal diagnosis of ectopia cordis j ultrasound med 3:429-31