A 20-year-old male was referred for echocardiography after presenting to his GP with tonsillitis. A new loud pansystolic murmur was detected. He was previously fit and well with a past medical history of thyrotoxicosis treated with subtotal thyroidectomy and stable immune thrombocytopenia (ITP).

A Transthoracic Echocardiogram (TTE) and Transesophageal Echocardiogram (TOE) confirmed the diagnosis of a bicuspid aortic valve with severe aortic regurgitation. The aortic root was dilated, maximally 4.2cm. The left ventricle was dilated 5.9cm on echo and 7.0cm on a cardiac CT with a borderline low Ejection Fraction of 50%. It was therefore decided to refer and discuss at a congenital multidisciplinary team meeting. The main options discussed were aortic valve repair, aortic valve replacement and a Ross procedure. A CT scan was performed, and, due to discrepant valve sizes, valve replacement was deemed the only viable option for this patient. He then underwent a mechanical aortic valve replacement (CarboMedics supra-annular top hat 27mm). He recovered well; however, 9 months post-operation he presented with weakness and aphasia, and CT head showed a left Middle Cerebral artery infarct. He also underwent thrombectomy. It was noted that he had periods of subtherapeutic INR.

This case presentation highlights complications that can arise with valve replacement and durability of surgical operation versus the complications of lifelong anticoagulation. We review the different surgical options available for severe aortic regurgitation, especially in the younger, congenital cohort of patients. This case increases awareness of the possibility of referring to congenital centers for the consideration of the Ross procedure.