Case Review Session 3: Valvular Heart Disease and Flow (2020)-Atresia of the coronary sinus ostium with persistent left vitelline vein connecting to the IVC; an unusual cause of a left-to-right shunt.

Atresia of the coronary sinus ostium with persistent left vitelline vein connecting to the IVC; an unusual cause of a left-to-right shunt.

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Video Transcription

Video Summary

A woman with dyspnea and progressive exercise limitation underwent cardiac MRI for suspected pulmonary hypertension. Imaging showed right atrial and ventricular dilation, severe tricuspid regurgitation, dilated hepatic veins/IVC, and normal pulmonary veins. A rare venous channel from the left atrium to the left hepatic vein was identified and ultimately diagnosed as a persistent left vitelline vein. It connected with an unroofed coronary sinus and atretic coronary sinus ostium, creating a significant left-to-right shunt (Qp:Qs 1.7). Embryology explained the defect. Intervention was offered, but the patient chose medical follow-up.

Keywords

persistent left vitelline vein

unroofed coronary sinus

left-to-right shunt

cardiac MRI

pulmonary hypertension