1.Aortic Regurgitation secondary to RCC prolapseDr Hannah ZR McConkey Cardiology Specialist Registrar Dr Laszlo Halmai Consultant Cardiologist Dr Bernard Prendergast Consultant CardiologistMilton Keynes and Oxford UK

2. Medical History 38 year old man No significant past medical history, including rheumatic fever Referred to cardiology outpatient clinic with a history of dyspnoea for several weeks Non smoker No alcohol No cardiac family history 3. On Examination Well Physical examination BP 128/55mmHg HR 74 beats / min No signs of congestive heart failure Collapsing pulse Diastolic murmur ECG 4. 2D Transthoracic Echocardiography Parasternal long axis and parasternal short axis views Suspicious bicuspid aortic valve with doming of the cusps 5. 2D Transthoracic Echocardiography M Mode 6. 2D Transthoracic Echocardiography 3 chamber view showing eccentric jet of severe aortic regurgitation (AR) resulting in restricted movement of the anterior mitral valve leaflet Severely dilated left ventricle (130mls/m2) with well preserved systolic functionRVRA 7. 2D Transthoracic EchocardiographyDiastolic flow reversal in the descending aorta 8. Management Cardiac Catheterisation Normal coronary arteries LVEDP 30mmHg Aortogram: normal ascending aorta and arch, with severe AR filling the left ventricle within one heart cycle Preserved left ventricular (LV) systolic function Transoesophageal Echocardiogram Moderate -severe LV dilatation with mildly impaired global systolic function The right coronary cusp appears to prolapse on 3D imaging with resulting very eccentric, anteriorly directed regurgitation restricting the opening of the anterior mitral valve leaflet 9. Transoesophageal Echocardiogram 10. Transoesophageal Echocardiogram 11. 3D Transoesophageal Echocardiography 12. 3D Transoesophageal EchocardiographyWatch video 13. Treatment: Aortic Valve Replacement Native aortic valve Tricuspid 3 commissures Fusion of the left and right cusps Redundant prolapsing right cusp Repair attempted Opening of fused commissure Augmentation of valve leaflets at each commissure Plication of redundant tissue at mid-point of right cusp TOE result poor - valve replaced with 25mm Medtronic mechanical prosthesis 14. Diagnosis:Aortic regurgitation secondary to pseudo-bicuspid aortic valve with prolapsing right coronary cusp 15. Aortic valve prolapse Aortic regurgitation can result from either leaflet dysfunction or dilatation of any component of the annulus Leaflet dysfunction can be described as type 2 (leaflet prolapse) or type 3 (leaflet restriction)1 Cusp prolapse is strictly defined as motion of the cusp free margin below the level of the middle of the sinuses of Valsalva Prolapse of the right coronary or non-coronary cusps is significantly more common than of the left coronary cusp2 Leaflet prolapse is more common in bicuspid valves31. 2. 3.Boodhwania M, de Kerchoveb L, Glineurb D, Noirhommeb P, El Khouryb, 2009. Repair of aortic valve cusp prolapse. MMCTS 2009, Issue 0702 Price J, De Kerchove L, El Khoury G, 2011. Aortic valve repair for leaflet prolapse. Semin Thorac Cardiovasc Surg 23(2):149-51 El Khoury G, Vanoverschelde JL, Glineur D, et al., 2006. Repair of bicuspid aortic valves in patients with aortic regurgitation. Circulation 2006;114(1 Suppl.):I610-I616 16. Echocardiographic Features of Cusp Prolapse1 Eccentric aortic regurgitant jet in the opposite direction of the prolapsing cusp Visualization of the valve cusp below the level of the aortic annulus during diastole Diminished length of aortic leaflet coaptation 17. Repair-Oriented Functional Classification of Aortic Insufficiency1 18. Leaflet Prolapse Repair with Central Plication4. de Kerchove L et al, 2008. Eur J Cardiothorac Surg;34:785-791 19. TAKE HOME MESSAGES Aortic leaflet prolapse can arise in bicuspid and tricuspid aortic valves Bicuspid aortic valves are more commonly affected Eccentric jets of aortic regurgitation may be difficult to evaluate and severity is easily under-estimated Transoesophageal echocardiography is key to determining the severity of aortic regurgitation and underlying mechanism Aortic valve repair may be attempted in experienced centres and by an experienced surgeon but the adequacy of the result must be checked intra-operatively 20. Join the ESC Working Group on Valvular Heart Disease and take part in its activities !Membership is FREE!