Double outlet right ventricle with persistent left superior vena cava into coronary sinus (prenatal ultrasound)

DORV double outlet right ventricle = occurs when both great arteries arise entirely or predominantly from the RV.

Several types of DORV exist according to the location of the VSD, the spatial relationship of the great arteries and whether or not there is an outflow obstruction.

Pulmonary stenosis is the most common associated cardiac anomaly.

Chromosomal anomalies are found in some fetuses (12% up to 40%) such as trisomies 18,13 and 22q1.1 microdeletion (region in the long arm (q) of chromosome 22).

Tetralogy of Fallot and Transpositiontransposition of the great arteries are two main differential diagnoses for DORV. 

Subpulmonic VSD and aortic coarctation are associated with suboptimal postsurgical outcomes in DORV.

Heart US examination at 1st trimester is limitedHere, however some elements can be examinated such as heart location in the thorax and heart axis.Color doppler is also used to assess anterograde chamber filling and anterograde blood ejection through the output vessels.Here, heart location and axis are normal.

1st pic image: 4 chamber view showing 2 ventricles with anterograde filling.

2nd pic image: outflow tracts. The expected pattern would be a "V-shaped" image representing the ductus arteriosus joining the aorta. Instead of that, oddly here, there is only one big tube showing an anterograde flow.

Levocardy, Situs solitus.

Atrioventricular concordance, well balanced LV and RV, normal atriventricular valves.

Perimembranous subaortic VSD, left-to-right shunt.

Malposition of the great arteries with both vessels arising from the RV : dextroposed aorta and left-sided aortic arch, smaller pulmonary trunk with normal left PA and right PA. Both great arteries have anterograde flow without stenosis, anterograde ductus arteriosus, which means this is not a ductus-dependant condition at birth.That is the description of a double outlet right ventricle, with subaortic VSD (the so called-called Fallot type).There is also a left persistent superior vena cava draining into the enlarged coronary sinus.

Thymus is present (absence of thymus may suggest a 22q1.1 microdeletion aka DiGeorge Syndrome).See annotated images. Some legends are in englishEnglish because I have added them later to upload the case, some legends are in french because I hadFrench as they were added them live so here's aduring scanning. A quick translation: CIV = VSD, AP=PA. BIF = bifurcation.