Coronary sinus atresia – congenital and acquired

Coronary sinus atresia – congenital and acquired

Coronary sinus atresia – congenital and acquired:

Abstract: Coronary sinus atresia is usually a congenital anomaly. Sometimes it is acquired, due to an AV fistula involving the middle cardiac vein which becomes grossly enlarged and impinges on the coronary sinus ostium and closes it off.

Congenital atresia of the right atrial ostium of the coronary sinus can lead to different pathways for drainage of coronary venous outflow. In some cases, coronary venous outflow may move through a persistent left superior vena cava into the left brachiocephalic vein. From the left brachiocephalic vein it reaches the right atrium through the right superior vena cava.^1,2 In another case this pattern was associated with additional aortic arch hypoplasia and ventricular septal defect.³ Rare instances in which the coronary venous blood was diverted to the left atrium has also been described.⁴ It is usually through an unroofed coronary sinus which opens into the left atrium.⁵

In this era of cardiac resynchronization therapy, failure to cannulate the coronary sinus for left ventricular lead implantation often prompts the detection of atresia of the right atrial ostium of the coronary sinus. Levophase of left coronary angiogram shows the coronary sinus ostial atresia and the track of coronary venous drainage. Implantation of the left ventricular lead through the persistent left superior vena cava is then resorted to.⁶ In this case there was also a large thrombus at the junction of the persistent left superior vena cava and the coronary sinus.
Coronary sinus atresia was detected in one case during coronary angiography prior to surgical repair of an atrial defect in a pentagenerian.⁷ In this case also there was an associate persistent left superior vena cava draining to the left innominate vein. From the left innominate vein, coronary outflow reached the right atrium through the right superior vena cava. Atresia of the coronary sinus ostium into the right atrium was further confirmed at surgical repair of the atrial septal defect.

References

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