Right coronary artery originating from the left ventricular outflow tract diagnosed after a Ross procedure: a case...

Anomalous coronary origin from the left ventricular outflow tract (LVOT) is an exceedingly rare condition thought to be associated with the bicuspid aortic valve (BAV). While the malignant presentation of this entity has been described, its pathophysiology and diagnostic evaluation are poorly understood.

A 33-year-old woman status post Ross procedure in childhood for congenital aortic stenosis due to BAV with presumed common origin of right and left coronary arteries based on single coronary ostium seen on aortic valve inspection, presented with symptomatic pulmonary regurgitation and stenosis. Invasive left coronary angiography revealed retrograde filling of the right coronary artery (RCA) with systolic washout of contrast indicating a patent RCA ostium. No RCA ostium was found on aortic root injection, but an injection into the LVOT revealed an RCA ostium below the aortic valve. Selective RCA angiography revealed pulsatile antegrade flow down the RCA occurring during systole. There was no anatomic RCA stenosis. We proceeded with valve-in-valve TcPVR. The patient had significant improvement of symptoms and RCA reimplantation was hence deferred.

This case is the first of an anomalous coronary artery arising from the LVOT diagnosed in a patient after the Ross procedure. Our angiograms shed light on the unusual physiology of coronary filling during systole and ischaemia arising from inadequate perfusion gradient between the left ventricle and the coronary during systole, leading to collateralization despite the lack of anatomic stenosis. We urge consideration of this potentially malignant entity in any symptomatic patient, especially with concomitant BAV.