Sameh Abdel Latif Abdel Salam, M.Sc.¹; Islam Badr, M.Sc.²; Mahmoud Alalfy, M.Sc.³

1. Radiodiagnosis department, Kasr Alainy teaching hospitals, Cairo university, Egypt;
2. Fetal medicine unit, Cairo university, Egypt;
3. Obstetric and gynecology department, National research Center, Egypt..

28-year old woman, G3, P2 was referred to our office for complete cardiac assessment due to history of previous death of her two babies due to cardiac defects. 

Ultrasound examination at 31 weeks gestation revealed the following findings:

- Single ventricle of left morphology is seen connected only to the right atrium. The small left atrium is seen receiving both inferior pulmonary veins and drains into the right atrium through patent foramen ovale with absent left atrio ventricular connection. The larger right atrium is connected to the single ventricle through tricuspid valve with evident tricuspid regurgitation and myocardial hypertrophy of its trabecular part.

- Both great vessels arise from the single ventricle with normal relationship in the mediastinum. The aorta is seen extremely small in caliber with no forward flow within. Retrograde flow in the hypoplastic aortic arch is seen.

- Patent pulmonary valve, main pulmonary artery and both central pulmonary arteries.

- The right superior pulmonary vein is seen coursing from right lung hilum towards the midline filling into its fellow from the left side forming a vertical vein that courses anteriorly to drain into posterior aspect of right superior vena cava (SVC).

- Evidence of persistent left superior vena cava draining into aneurysmally dilated coronary sinus.

- Bidirectional flow is seen within the dilated coronary sinus through widely opened coronary sinus ostium and incompetent thebesian valve.

- Bradycardia (FHR=98bpm).


Conclusion

Early severe aortic arch coarctation or tubular hypoplasia causes aortic atresia with retrograde flow within the aortic arch.

Single ventricle with absent left atrio-ventricular connection.

Persistent left superior vena cava draining into aneurysmally dilated coronary sinus.

Partial anomalous pulmonary venous connection of both superior pulmonary veins into vertical vein draining into right superior vena cava.

A male neonate was born at 37 weeks gestation. Severe respiratory distress was present. IV infusion of prostaglandin was started but unfortunately the baby died due to severe respiratory distress with failure of all methods of resuscitation.

Images 3, 4, 5, videos 2, 3: Color Doppler study shows tricuspid regurgitation, bidirectional flow through dilated coronary sinus, inflow of both inferior pulmonary veins into LA, flow shunt form left atrium into right atrium through patent foramen ovale, retrograde flow in aortic arch.

Images 6, 7, 8, videos 4, 5: show prominent main pulmonary artery, central pulmonary arteries and ductus arteriosus with aortic atresia. Persistent left SVC is seen to the left of pulmonary artery.

Images 9, 10 and video 6: show the anomalous connection of both superior pulmonary veins into vertical vein filling into posterior aspect of right superior vena cava (SVC).

Image 11: pulsed Doppler study of the main pulmonary artery performed in short axis view showing fetal bradycardia with HR = 98 bpm denoting evidence of heart block.