Abstract 4137869: Ross Procedure for Correction of Congenitally Unicuspid Aortic Valve: A Paradigm Shift?

Case 1: A 54-yr-old man reported exertional dyspnea for 3 months, with a systolic murmur on exam. TTE noted severe aortic stenosis, moderate insufficiency and possible bicuspid valve morphology. Coronary angiogram showed no obstructive disease. CT chest showed a 5-cm ascending aortic aneurysm. CT surgery evaluation was sought, and patient had a Ross procedure with aortic hemiarch repair. Intra-operative TEE confirmed a congenitally unicommissural, unicuspid aortic valve (UAV). A 7-month follow-up TTE showed a normal aortic and pulmonic prosthetic gradient. Case 2: A 46-yr-old lady reported progressive exercise intolerance, with a systolic murmur and faint S2 noted on exam. TTE showed severe aortic stenosis. MR angiogram showed a unicuspid valve and a mildly dilated aortic root. The patient was evaluated by CT surgery and underwent Ross procedure. TTE 4 years later showed a normal aortic and a moderately elevated pulmonic prosthetic gradient. Patient continues to exercise without limitations. Discussion: UAV is a rare entity with an incidence of 0.02%. It has a bimodal distribution, in infants and adults, with the unicommissural type more common in adults. Due to rapid progression of valvular disease, the mean age at diagnosis is 42 years. Concurrent aortic dilatation is present in 48% patients. Management of UAV is a matter of ongoing investigation. Classically, young patients with aortic valve disease underwent mechanical AVR. Ross procedure was under-utilized due to concerns about the surgical complexity and potential need for re-operation of the aortic and pulmonic grafts. However, recent studies show promising results. Patients with UAV have lower reoperation rates after a Ross procedure compared to a repair alone. Furthermore, a Ross procedure confers mortality benefit over mechanical AVR with no significant difference in immediate perioperative complications and mortality, although, this needs further investigation in a UAV cohort. Our cases support the feasibility of a Ross procedure for UAV, in providing a durable solution while avoiding anticoagulation in young patients. As Ross procedure is performed in limited centers, patients with UAV should be referred to such centers for definitive management.