Complex congental cardiac malformation (prenatal ultrasound)

Case contributed by Fabien Ho


Standard risk pregnancy, no peculiar familial history, no peculiar obstetrical history. Isolated, non syndromic finding on routine exam.

Patient Data

Age: Fetus
Gender: Male

Description of the videos in their displaying order left to right:
1: first impression: large VSD, unbalanced ventricles, output vessels do not cross each other.
2: output vessels do not cross each other.
3: situs - stomach on the left, cardiac axis on the right, the RV being anterior: dextrocardia.
4: AV connection: pulmonary veins connecting to a left atrium. No right atrium is difficult to identify. One AV valve connecting to a left-type main ventricle.
5: Focus on the large VSD between the left-type ventricle and the small right-type ventricle. In situations with a functionally univentricular heart, the large VSD is called "bulbo ventricular foramen".
6: Focus on outflow tracts: 2 large outflow tracts, each with their own valves, parallel to each other, arising from an equivalent of unique functional ventricle (left-type as main, and large VSD opening in archaic right-type one).
7: Doppler on outflow tracts: both anterograde flow in both, without aliasing. Back to video 2- on axial plane, the anterior vessel does not divide itself: an aorta, which is right-sided. The middle vessel divides itself early: pulmonary trunk.
8, 9, 10: careful examination reveals that the ductus arteriosus feeds the descending thoracic aorta alone. No connection between aortic isthmus after subclavian left artery: interrupted aortic arch type A.
11. systemic venous return with IVC connecting to the right atrium (OD).

Focus on outflow tracts at 31WG :
1: Axial dynamic scanning shows 2 outflows tracts: one smaller, non-dividing, arising from the small right-type ventricle: the aorta. The other larger with early division, in middle position, arising from the main left-type ventricle : the pulmonary trunk.
2: Focus on the pulmonary trunk, both arteries and ductus have a large diameter. 

Case Discussion

The unique combination of heterotaxy, an equivalent of a univentricular heart, TGA and interrupted aortic arch makes this case unclassifiable.

This is a complex cardiac malformation because anatomical repair is not achievable, as this is an equivalent of a univentricular heart. The main functional ventricle is a left-type one, which is better to stand systemic pressure on the long term. Ductus arteriosus must remain open after birth, because it feeds descending thoracic aorta in this case.

It is of paramount importance to rule out associated anomalies which may complicate the overall prognosis, such as Down syndrome and DiGeorge syndrome. 

Courtesy of both Dr F. Cuillier and Dr L-A. Arsac, who both significantly contributed to this case.

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