738 A RARE CASE OF UNICUSPID AORTIC VALVE WITH SEVERE STENOSIS AND REGURGITATION: CLINICAL PRESENTATION AND MANAGEMENT


 A 19-year-old Pakistani migrant, who arrived in our country on foot, presented at emergency department with angina and worsening dyspnoea on exertion. The patient reported an unclear history of aortic valve disease.
 Clinical examination revealed a 6/6L systolic murmur with thrill. The electrocardiogram showed sinus rhythm and signs of left ventricular hypertrophy. Chest X-ray reported cardiomegaly and signs of congestion. Troponin I HS was mildly elevated (39 ng/L, n.v. < 18 ng/L).
 The patient was admitted to our Cardiology department. Transthoracic echocardiography revealed a mildly dilated left ventricle (LV), with severe hypertrophy, normal kinetics and systolic function (EF 63%), dilated ascending aorta (diam. 41 mm) and severe aortic steno-insufficiency. Transesophageal echocardiography was then performed, revealing a highly calcified, unicuspid unicommissural aortic valve with severe stenosis and regurgitation. A separate origin of left coronary ostia was also documented.
 The patient was referred to urgent cardiac surgery. A modified Bentall procedure (mechanical valve and prosthetic conduit) was performed with reimplantation of the coronary ostia. Due to a post-surgical complete atrioventricular block, a dual-chamber pacemaker (DDD) was implanted.
 Follow-up echocardiography showed reduction of LV volumes and wall thickness and normal prosthetic function.
 Unicuspid aortic valve is a very rare congenital disease, with a prevalence of about 0.02% in the adult population and is frequently associated with ascending aorta dilatation. The treatment is aortic valve replacement. Complete atrioventricular block can occur after surgery, requiring cardiac pacing.