Bicuspid Aortic Stenosis (New Surgical Approaches) Joseph S. Coselli, M.D. Professor and Cullen Foundation Endowed Chair Division of Cardiothoracic Surgery Baylor College of Medicine Heart Valve Summit 2013 Session II: New Treatment Approaches Chicago, IL • Thursday, September 26, 2013 Conflict of Interest Medtronic, Inc. Vascutek Terumo WL Gore & Associates Research Support, Honorarium, Consultant, Speaker Educational Grant, Consultant, Royalties, Speaker Research Support, Advisory Board Calcified Bicuspid Aortic Valves Images: Heart-Valve-Surgery, Library.Med.Utah, Ho 2008 EJ Echo, Robertson 2012 What’s new in surgical approaches to bicuspid aortic stenosis? WTF? What’s new in surgical approaches to bicuspid aortic stenosis? **$@!!@! Bicuspid Aortic Valves • Common (1-2% of population) • A third → valve complication • A third to half → aortic dilatation Tricuspid AV Bicuspid AV Bicuspid Aortic Valves Type II TAV Type III Type I Most Common ~80% ~1% • Common (1-2% of population) 3-6 million US citizens affected (males 4:1) Congenital or acquired (functional) • A third → valve complication • A third to half → aortic dilatation Images from Abdulkarrem 2013 ICVTS Bicuspid Aortic Valves • Common (1-2% of population) • A third → valve complication Aortic valve stenosis (AS) most common valve complication Aortic valve insufficiency (AI) AS mixed with AI None: functionally normal • A third to half → aortic dilatation Bicuspid Aortic Valves • Common (1-2% of population) • A third → valve complication • A third to half → aortic dilatation Ascending aorta is often affected With and without involving the aortic root Aortic arch may be additionally affected Distal aorta rarely affected Varying patterns of dilatation 13% 14% 28% 45% Great heterogeneity Fazel 2008 JTCVS Heterogeneity: A Tale of 2 Brothers with BAV Stenosis Rapid progression of symptoms during last month of life •breathlessness •dizziness •peak gradient of 56 mmHg •mean gradient of 39 mmHg •AVA 0.6 cm2 •Repair performed, but he died 2 weeks later Normal Deceased Age 16 Deceased Age 21 No evidence of cardiac dysfunction. Dissection following amphetamine use •5 cm tear •dilated ascending aorta •severe coarctation •normally functioning valve •leaflets soft and pliable •Found dead Variability Within Same Family Zafar Proc BUMC 2013;26:171-173 Case report of 2 brothers with congenital BAV Aortopathy Associated with Complications of Aortic Valve Stenosis Supracoronary Aneurysm Insufficiency Annuloaortic Ectasia (Marfan Syndrome) Both Tubular Diffuse Enlargement Differing Embryogenesis • The ascending aorta and aortic arch are derived from Neural Crest Cells • The distal aorta is derived from the Mesoderm The descending aorta in a murine model is indicated to be free of neural crest cells, unlike the aortic root and arch. Bergwerff Circ Res 1998 Leroux-Berger JBMR 2011 [France] Nakamura Circ Res 2006 Many BAV Associated Genes Multiple genes associated with BAV; Decreased penetrance of BAV Abdulkareem ICVTS 2013 Many BAV Associated Genes Not a single-gene mutation! Greatly enhances complexity Most mutations are sporadic (91%) A few mutations are familial (~9%) Multiple genes associated with BAV; Variable penetrance of BAV Abdulkareem ICVTS 2013 Driver of BAV Aortopathy? Controversy!!! Flow hypotheses • Hemodynamic flow • Tissue fundamentally disturbance different from normal weakens aortic tissue, not unlike aorta Marfan syndrome • Turbulent • Ascending aorta blood flow susceptible to mutation in passing neural crest (cell origins) through valve affects ascending aorta Genetic abnormality Plaisance 2012 Aortic Tissue Properties → BAVs • Medial degeneration with loss of elastic fibers • Loss and reorientation of smooth muscle cells • Less type I collagen • Fibrillin -1 deficiency Normal Aorta • • • Extracellular matrix disrupted MMP increased, ↑degradation ECM proteolytic cascade altered Tamarina 1997 Surgery de Sa 1999 JTCVS Bauer 2002 ATS BAV Aorta Della Corte et al ▪ J Heart Valve Dis 2006 Fedak et al ▪ Circulation 2002 LeMaire J Surg Res 2005 Bicuspid Tricuspid MMP-2 (Immunohistochemical staining) • MMP-2 Significantly increased in BAV LeMaire J Surg Res 2005 Control Bicuspid Tricuspid MMP-9 (Immunohistochemical staining) • MMP-9 Significantly decreased in BAV LeMaire J Surg Res 2005 Control BAV: Aortic Stenosis • Some inherent dysfunction • In adults, most AS is due to leaflet calcification as bicuspid valve opens • Often calcification is abnormally even without rapid and severe symptoms Normal valve opens & closes quickly. Fibrosis/calcification are common Fusion of the right and left cusp Voigt ▪ 2010 ; Ionasec ▪ 2010 Images: Roberts Medicine 2012 BAV Calcification: Macrophage Infiltration Bicuspid • Mechanism for rapid progression of BAV stenosis? • Neovascularizaion and macrophage infiltration > BAV Inflammation (P = .002) Bicuspid Tricuspid Tricuspid Neovascularization (P = .0005) Bicuspid Tricuspid Moreno 2011 JTCVS; 142:895-901; Comparison of excised leaflets of stenotic TAV and BAV BAV: Aortic Dissection Most significant potential complication in BAV is acute ascending dissection 8-fold increased risk! Michelena JAMA 2011;306:1104-12 • BAV patients with AS are at far greater risk of aortic dissection than TAV patients 86 Mayo Clinic patients with type A dissection 19% BAV have ≥ 40 mmHg mean gradient 0% TAV have ≥ 40 mmHg mean gradient Eleid Heart 2013 in press 288 Chinese patients with type A dissection 23% BAV have > 25 mmHg mean gradient 0% TAV have > 25 mmHg mean gradient Wang EFCTS 2013;44:172-77 Type A dissection at RCA in patient with AS and endocarditis Non-Right cusp fusion Iwasa ICVTS 2011 Aortic Dilatation in BAV Stenosis Ascending Significant Subset of patients with Severe AS Ascending Significant Subset of patients with Mild/mod AS Patients with stenotic BAVs have greater dilation of the ascending aorta than do stenotic TAV patients • Dilatation ≥ 5.0cm was 35% in BAV and 4% in TAV 84 BAV pts vs 103 TAV pts Debl 2009 Clin Res Cardiol BAV: Aortic Stenosis • BAV is present in about 50% of all adult cases of aortic valve stenosis • Process is similar to senile degenerative AS but happens decade earlier in BAV patients 52% of all cases of AS in patients < 80 years Roberts Am J Cardiol2012;109:16321636 Patients from 1993 to 2011 with excised native stenotic aortic valves (with or itho t AI) 1725 patients Treatments for Aortic Stenosis Treatment of predominantly or purely calcific valve stenosis has always been problematic Investigated Treatments •Decalcification Open manual debridement, surgical valvuloplasty, sculpturing (1950s-60s) Laser/ultrasonic debridement (1980s-2000s) • Dental plaque fragmentizers • Urologic instruments for kidney stones • Cavitron surgical aspirator (CUSA) Fix it? Ultrasonic Decalcification • • • • Investigated as treatment for calcification/stenosis Late insufficiency due to retraction of cusps Tendency towards recalcification of leaflets Technique largely abandoned Williamson Lasers Surg Med 1993;13:421-8. Dahm J Heart Valve Dis 2000;9:21-6. Kellner EJCTS 1996;10:498-504. Freeman JACC 1990:16:623-30. Expert Guidelines for Valvular Heart Disease In Setting of BAV Condition of ascending aorta/aortic root > 5.0 cm Fast growing (≥ 0.5 cm per year) Concomitant repair of valve And ascending aorta/root > 4.5 cm Patient is of small stature Same as 2006 guidelines Bonow 2008 JACC Guidelines Replace aorta Replace aorta Replace aorta Consider lowered threshold Class I Level C Recommendations Expert Aortic Guidelines Bicuspid aortic valve recommendations Condition of ascending aorta/aortic root 4.0 ↔ 5.0 cm Fast growing (≥ 0.5 cm per year) Concomitant repair of valve And ascending aorta/root > 4.5 cm Guidelines Replace aorta Replace aorta Replace aorta Class I Level C Recommendations Hiratzka 2010 Circulation European Heart Journal (2012) 33, 2451–2496 doi:10.1093/eurheartj/ehs109 ESC/EA CT S GU IDELIN ES Guidelines on the management of valvular heart disease (version 2012) T he Joint Task For ce on t he Managem ent of Valvular Hear t Disease of t he Eur opean Societ y of Car diology (ESC) and t he Eur opean A ssociat ion for Car dio-T hor acic Sur ger y (EACT S) Aut hor s/T ask For ce Mem ber s: Alec Vahanian (Chair per son) (Fr ance) * , Ot t avio Alfier i (Chair per son) * (It aly), Felicit a Andr eot t i (It aly), Manuel J. A nt unes (Por t ugal ), Gonzalo Bar ón-Esquivias (Spain), Helm ut Baum gar t ner (Ger m any), Michael Andr ew Bor ger (Ger m any), T hier r y P. Car r el (Swit zer land), Michele De Bonis (It aly), A r t ur o Evangelist a (Spain), Volkm ar Falk (Swit zer land), Ber nar d Iung (Fr ance), Pat r izio Lancellot t i (Belgium ), Luc Pier ar d (Belgium ), Susanna Pr ice (UK), H ans-Joachim Schäfer s (Ger m any), Ger har d Schuler (Ger m any), Janina St epinska (Poland), Kar l Swedber g (Sweden), Johanna T akkenber g (T he N et her lands), U lr ich O t t o Von O ppell (U K), St ephan W indecker (Swit zer land), Jose Luis Zam or ano (Spain), Mar ian Zem bala (Poland) Expert Valvular Guidelines In Setting of BAV Condition of ascending aorta/aortic root Guidelines regardless of valvular disease ≥ 50 mm with additional risk factors Consider surgery Risk factors: coarctation of the aorta, systemic hypertension, family history of dissection or increase in aortic diameter > 2 mm/year* ESC Com m it t ee for Pract ice Guidelines (CPG): Jer oen J. Bax (Chair per son) (The N et her lands), H elm ut Baum gar t ner (Ger m any), Claudio Ceconi (It aly), Ver onica Dean (Fr ance), Chr ist i Deat on (UK), Rober t Fagar d (Belgium ), Chr ist ian Funck-Br ent ano (Fr ance), David Hasdai (Isr ael ), Ar no Hoes (The N et her lands), Paulus Kir chhof (Unit ed Kingdom ), Juhani Knuut i (Finland), Philippe Kolh (Belgium ), T her esa McDonagh (UK), Cyr il Moulin (Fr ance), Bogdan A . Popescu (Rom ania), Željko Reiner (Cr oat ia), Udo Secht em (Ger m any), Per Ant on Sir nes (N or way), Michal Tender a (Poland), A dam Tor bicki (Poland), A lec Vahanian (Fr ance), St ephan W indecker (Swit zer land) Docum ent Reviewer s:: Bogdan A . Popescu (ESC CPG Review Coor dinat or ) (Rom ania), Ludwig Von Segesser (EACT S Review Coor dinat or ) (Swit zer land), Luigi P. Badano (It aly), Mat jaž Bunc (Slovenia), Mar c J. Claeys (Belgium ), N iksa Dr inkovic (Cr oat ia), Ger asim os Filippat os (Gr eece), Gilber t Habib (Fr ance), A . Piet er Kappet ein (T he N et her lands), Roland Kassab (Lebanon), Gr egor y Y.H. Lip (UK), N eil Moat (U K), Geor g N ickenig (Ger m any), Cat her ine M. Ot t o (USA ), John Pepper , (U K), N icolo Piazza (Ger m any), Pet r onella G. Pieper (T he N et her lands), Raphael Rosenhek (A ust r ia), N alt in Shuka (A lbania), Ehud Schwam m ent hal (Isr ael ), Juer g Schwit t er (Swit zer land), Pilar Tor nos Mas (Spain), Pedr o T. Tr indade (Swit zer land), T hom as W alt her (Ger m any) T he disclosur e for m s of t he aut hor s and r eviewer s ar e available on t he ESC websit e www.escar dio.or g/guidelines Online publish-ahead-of-print 24 August 2012 * Corresponding authors: Alec Vahanian, Service de Cardiologie, Hopital Bichat AP-HP, 46 rue Henri Huchard, 75018 Paris, France. Tel: + 33 1 40 25 67 60; Fax: + 33 1 40 25 67 32. Email: alec.vahanian@bch.aphp.fr Ottavio Alfieri, S. Raffaele University Hospital, 20132 Milan, Italy. Tel: + 39 02 26437109; Fax: + 39 02 26437125. Email: ottavio.alfieri@hsr.it †Other ESC entities having participated in the development of this document : Associations: European Association of Echocardiography (EAE), European Association of Percutaneous Cardiovascular Interventions (EAPCI), Heart Failure Association (HFA) Working Groups: Acute Cardiac Care, Cardiovascular Surgery, Valvular Heart Disease, Thrombosis, Grown-up Congenital Heart Disease Councils: Cardiology Practice, Cardiovascular Imaging The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal, and the party authorized to handle such permissions on behalf of the ESC. Disclaim er . The ESC/EACTSGuidelines represent the views of the ESC and the EACTSand were arrived at after careful consideration of the available evidence at the time they were written. Health professionals are encouraged to take them fully into account when exercising their clinical judgement. The guidelines do not, however, override the individual responsibility of health professionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient and, where appropriate and necessary, the patient’s guardian or carer. It is also the health professional’s responsibility to verify the rules and regulations applicable to drugs and devices at the time of prescription. & The European Society of Cardiology 2012. All rights reserved. For permissions please email: journals.permissions@oup.com Vahanian 2012 Eur Heart Journal *on repeated measurements using the same imaging technique, measured at the same aorta level with side-by-side comparison and confirmed by another technique Expert Aorta and Ascending Guidelines In Setting of BAV Condition of ascending aorta/aortic root > 5.0 cm Family history of dissection And > 4.5 cm Fast growing (≥ 0.5 cm per year) Concomitant cardiac repair And ascending aorta/root > 4.5 cm Guidelines Consider surgery Consider surgery Consider surgery Consider surgery Ratio ascending aortic diameter to height > 10 Without significant aortic root dilatation Consider surgery Consider Wheat Level B Recommendations Svensson Ann Thorac Surg 2013;95:S1-66 Expert Aorta and Ascending Guidelines Management of BAV Not Meeting Other Criteria All patients with BAV Level B evidence First-degree relatives of young patients with BAV Level C evidence Svensson Ann Thorac Surg 2013;95:S1-66 Guidelines Undergo imaging of thoracic aorta Advise further investigation Repair of Bicuspid Aortic Valves • Bicuspid valves are frequently repaired 32 of 374 pts (13%) David 2013 [Toronto] 17 of 55 pts (31%) Boodhwani 2011 [El Khoury] Entire series of 75 patients Kari 2013 [Stanford] Entire series of 153 patients Schäfers 2010[Saar] • When to consider repair Pliable leaflets Which BAVs can Minimal fibrosis and calcification be repaired rather No more than mild cusp thickening than replaced? Regurgitant valves (even if severe) Normally functioning valves Minor fenestrations Gleason STCVS 2006 Kari 2013 JTCVS (Stanford) 374 patients 13% BAV Freedom from Reoperation Balloon Valvuloplasty • Option for extreme-risk patients Retrograde orFunctionally antegrade bicuspid approach Major bleeding in about 20% Severity of AS reduced 30-40% Immediate symptomatic True bicuspid improvement No mid- or long-term benefit Maskatia 2013 Better outcomes in bicuspid? Aortic Valve Replacement Does not address aortopathy Standard techniques apply Wheat Operation Aortic valve replacement + supracoronary ascending aortic replacement (modified Wheat) Wheat ▪ JAMA 1964 Nazer ▪ ATS 2010 •Avoids reimplantation of coronaries •A good option in BAV stenosis when aortic root is not dilated Composite Valve Graft • Replacement of valve, root, and ascending Reimplantation of coronary arteries Mechanical, bio-valve, Preferred approach by many Bioroot • Option that avoids use of anticoagulants Reimplantation of coronary arteries Used alone, does not address extensive aortopathy May be combined with graft to extend repair Arch • Replacement of valve, root, ascending aorta, and hemi- or full arch Most comprehensive repair Enhanced risk Hypothermic circulatory arrest Homograft • Replacement of valve, root, and ascending Reimplantation of coronary arteries Lifespan somewhat unpredictable Roughly 10 to 12 years May be useful with concomitant infection or endocarditis Degenerated Homograft (replaced BAV--12 years postop) Degenerated Homograft (replaced BAV--12 years postop) Ross • Pulmonary autograft Infrequently used in adults Risk of late dilatation as pulmonary vessel shares embryonic source (neural crest) with proximal aorta Minimally Invasive Right infraaxillary thoracotomy (Ito 2013) Right anterior thoracotomy (Glauber 2011) Mini median thoracotomy (Alassar 2013) • Variety of minimally invasive approaches for isolated AVR Selective use in BAV AS Results may = standard AVR Venting heart may be difficult Mechanical or bio-valves Urgent conversion possible 3% Gilmanov 2013 2% Glauber 2011 Ito ATS 2013;96:715-7; 25 patients—17 with aortic stenosis, congential BAV in 5 pts; no early deaths Gilmanov ATS 2013;96:837-43; 182 RA and upper mini matched to conventional AV; 1.6% early death for both; 47% AS Glauber JTCVS 2011;142:1577-9. Alassar JCTS 2013; 8:103; 53 of 58 pts have AS; No early death in 58 patients Minimally Invasive Approach: isolated AVR Use in 192 patients Median EuroScore 5.2% Stenosis in 90 (47%) Biovalve in 160 (83%) Early death in 3 (1.6%) Conversion to sternotomy in 3 (1.6%) Blood transfusion in 31 (16%) Stroke in 1 (0.5%) Atrial fibrillation 35 (18%) Right anterior thoracotomy (Glauber 2011) Glauber JTCVS 2011;142:1577-9. [Italy] Minimally Invasive Approaches to isolated AVR 58 patients; 53 with AS (91%) STS risk score 5.7% Biovalve used in 57 (98%) No early death No stroke Reoperation for bleeding in 1 patient (2%) Mean hospital stay 6 days Mini median thoracotomy (Alassar 2013) Hamburg, Germany Alassar JCTS 2013; 8:103; 53 of 58 pts have AS; No early death in 58 patients; Not clear if BAV present in any patients J-incision Minimally Invasive • J-incision permits less invasive valve and proximal aortic repair Acquir ed Car diovascular Disease Johnston et al Outcomes of less invasive J-incision appr oach to aor tic valve sur ger y Douglas R. Johnston, MD,a Fernando A. Atik, MD,a Jeevanantham Rajeswaran, MSc,b Eugene H. Blackstone, MD,a,b Edward R. Nowicki, MD, MS,a Joseph F. Sabik III, MD,a Tomislav Mihaljevic, MD,aA. Marc Gillinov, MD,a BruceW. Lytle, MD,a and LarsG. Svensson, MD, PhDa ACD 8-10 cm incision CLEVELAND CLINIC Obj ective: Less invasive approaches to aortic valve surgery are increasingly used; however, few studies have investigated their impact on outcome. We sought to compare clinical outcomes after these approaches with full sternotomy using propensity-matching methods. M ethods: From January 1995 to January 2004, a total of 2689 patients underwent isolated aortic valve surgery, 1193 viaupper J-hemisternotomy and 1496 viafull sternotomy. Becauseof important differencesin patient characteristics between these groups, a propensity score based on 42 variables was used to obtain 832 well-matched patient pairs (70% of possible cases). Results: In-hospital mortality was identical for propensity-matched patients, 0.96% (8 in each). Occurrences of stroke (P> .9), renal failure (P ¼ .8), and myocardial infarction (P ¼ .7) were similar. However, 24-hour mediastinal drainage was a third less after less invasive surgery (median, 250 vs 350 mL; P< .0001), and fewer patients received transfusions (24% vs 34% ; P < .0001). More patients undergoing less invasive surgery were extubated in the operating room (12% vs 1.6% ; P< .0001), postoperative forced 1-second expiratory volume was higher (P ¼ .009), and fewer had respiratory failure (P ¼ .01). Early after operation, pain scores were lower (P< .0001) after less-invasive surgery and postoperative length of stay shorter (P< .0001). Propensity matched cohort Propensity matched patients (832) Death Stroke J-incision Full 8 (1%) 8 (1%) 11 (1%) 11 (1%) 202 (24%) 286 (34%) Conclusions: Within that portion of the spectrum of isolated aortic valve surgery where propensity matching was possible, minimally invasive aortic valve surgery had not only cosmetic advantages, but blood product use, respiratory, pain, and resource utilization advantages over full sternotomy, and no apparent detriments. Less invasive aortic valve surgery should be considered for most aortic valve operations. (J Thorac Cardiovasc Surg 2012;144:852-8) Supplemental material is available online. Earn CME credits at http://cme.ctsnetjournals.org Red blood cell transfusion P<.0001 From the Department of Thoracic and Cardiovascular Surgery,a Heart and Vascular Institute, and Department of Quantitative Health Sciences,b Research Institute, Cleveland Clinic, Cleveland, Ohio. This study was supported in part by the Kenneth Gee and Paula Shaw, PhD, Chair in Heart Research (Dr Blackstone), the Donna and Ken LewisChair in Cardiothoracic Surgery and Peter Boyle Research Fund (Tomislav Mihaljevic), and the Judith Dion Pyle Chair in Heart Valve Research (Dr Gillinov). Disclosures: Dr Mihaljevic isaconsultant for Edwards Lifesciencesand IntuitiveSurgical and receives speaker fees from Intuitive Surgical. Dr Gillinov is a consultant to Edward Lifesciences and receives honorari a for speaking from St Jude Medical, Inc. He has an equity interest in Viacor, Inc. R i df bli ti S t 15 2010 i i i d N 7 2011 t df In the mid-1990s, less invasive ‘‘ keyhole’’ approaches for valve operations were pioneered with the intent of reducing morbidity, postoperative pain, and blood loss, improving cosmesis, shortening hospital stay, and reducing cost compared with the 50-year-old full sternotomy approach.1-10 Furthermore, it was believed that less spreading of the incision, not interfering with the diaphragm, and less tissue dissection might improve outcomes, particularly respiratory function.7,8 Although clinical studies suggest that some of these benefits have been realized, there has been no confirmatory large study or randomized trial.1-10 Because patients undergoing aortic valve surgery are in general older and sicker than those undergoing isolated mitral valve surgery, cosmetic benefits of less invasive aortic valve surgery may not be as important. Yet potential improvement in postoperative pain and respiratory function, particularly in patients with advanced respiratory disease, and reduced blood loss, transfusion requirement, and intensive care unit (ICU) and hospital lengths of stay are of even greater possible benefit in this older population Johnston JTCVS 2012;144:852; valve dysfunction not clear; < 3% of cases were converted to full sternotomy US (NIS) Practice Trends for BAV For AVR+ Aorta Substantial Increase 50% get mechanical valves Opotowsky JTCVS 2013;146:339-46 ~50,000 patients; for all procedures, use of mechanical valves decreased from 69% to 38% (1998-2009) Information of type of pathology is not available (mix of AS, AI, normal functioning valves) Anything Else? European Heart Journal (2012) 33, 2451–2496 doi:10.1093/eurheartj/ehs109 ESC/EA CT S GU IDELIN ES Guidelines on the management of valvular heart disease (version 2012) T he Joint Task For ce on t he Managem ent of Valvular Hear t Disease of t he Eur opean Societ y of Car diology (ESC) and t he Eur opean A ssociat ion for Car dio-T hor acic Sur ger y (EACT S) Aut hor s/T ask For ce Mem ber s: Alec Vahanian (Chair per son) (Fr ance) * , Ot t avio Alfier i (Chair per son) * (It aly), Felicit a Andr eot t i (It aly), Manuel J. A nt unes (Por t ugal ), Gonzalo Bar ón-Esquivias (Spain), Helm ut Baum gar t ner (Ger m any), Michael Andr ew Bor ger (Ger m any), T hier r y P. Car r el (Swit zer land), Michele De Bonis (It aly), A r t ur o Evangelist a (Spain), Volkm ar Falk (Swit zer land), Ber nar d Iung (Fr ance), Pat r izio Lancellot t i (Belgium ), Luc Pier ar d (Belgium ), Susanna Pr ice (UK), H ans-Joachim Schäfer s (Ger m any), Ger har d Schuler (Ger m any), Janina St epinska (Poland), Kar l Swedber g (Sweden), Johanna T akkenber g (T he N et her lands), U lr ich O t t o Von O ppell (U K), St ephan W indecker (Swit zer land), Jose Luis Zam or ano (Spain), Mar ian Zem bala (Poland) ESC Com m it t ee for Pract ice Guidelines (CPG): Jer oen J. Bax (Chair per son) (The N et her lands), H elm ut Baum gar t ner (Ger m any), Claudio Ceconi (It aly), Ver onica Dean (Fr ance), Chr ist i Deat on (UK), Rober t Fagar d (Belgium ), Chr ist ian Funck-Br ent ano (Fr ance), David Hasdai (Isr ael ), Ar no Hoes (The N et her lands), Paulus Kir chhof (Unit ed Kingdom ), Juhani Knuut i (Finland), Philippe Kolh (Belgium ), T her esa McDonagh (UK), Cyr il Moulin (Fr ance), Bogdan A . Popescu (Rom ania), Željko Reiner (Cr oat ia), Udo Secht em (Ger m any), Per Ant on Sir nes (N or way), Michal Tender a (Poland), A dam Tor bicki (Poland), A lec Vahanian (Fr ance), St ephan W indecker (Swit zer land) Docum ent Reviewer s:: Bogdan A . Popescu (ESC CPG Review Coor dinat or ) (Rom ania), Ludwig Von Segesser (EACT S Review Coor dinat or ) (Swit zer land), Luigi P. Badano (It aly), Mat jaž Bunc (Slovenia), Mar c J. Claeys (Belgium ), N iksa Dr inkovic (Cr oat ia), Ger asim os Filippat os (Gr eece), Gilber t Habib (Fr ance), A . Piet er Kappet ein (T he N et her lands), Roland Kassab (Lebanon), Gr egor y Y.H. Lip (UK), N eil Moat (U K), Geor g N ickenig (Ger m any), Cat her ine M. Ot t o (USA ), John Pepper , (U K), N icolo Piazza (Ger m any), Pet r onella G. Pieper (T he N et her lands), Raphael Rosenhek (A ust r ia), N alt in Shuka (A lbania), Ehud Schwam m ent hal (Isr ael ), Juer g Schwit t er (Swit zer land), Pilar Tor nos Mas (Spain), Pedr o T. Tr indade (Swit zer land), T hom as W alt her (Ger m any) T he disclosur e for m s of t he aut hor s and r eviewer s ar e available on t he ESC websit e www.escar dio.or g/guidelines Online publish-ahead-of-print 24 August 2012 * Corresponding authors: Alec Vahanian, Service de Cardiologie, Hopital Bichat AP-HP, 46 rue Henri Huchard, 75018 Paris, France. Tel: + 33 1 40 25 67 60; Fax: + 33 1 40 25 67 32. Email: alec.vahanian@bch.aphp.fr Ottavio Alfieri, S. Raffaele University Hospital, 20132 Milan, Italy. Tel: + 39 02 26437109; Fax: + 39 02 26437125. Email: ottavio.alfieri@hsr.it †Other ESC entities having participated in the development of this document : Associations: European Association of Echocardiography (EAE), European Association of Percutaneous Cardiovascular Interventions (EAPCI), Heart Failure Association (HFA) Working Groups: Acute Cardiac Care, Cardiovascular Surgery, Valvular Heart Disease, Thrombosis, Grown-up Congenital Heart Disease Councils: Cardiology Practice, Cardiovascular Imaging The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal, and the party authorized to handle such permissions on behalf of the ESC. Disclaim er . The ESC/EACTSGuidelines represent the views of the ESC and the EACTSand were arrived at after careful consideration of the available evidence at the time they were written. Health professionals are encouraged to take them fully into account when exercising their clinical judgement. The guidelines do not, however, override the individual responsibility of health professionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient and, where appropriate and necessary, the patient’s guardian or carer. It is also the health professional’s responsibility to verify the rules and regulations applicable to drugs and devices at the time of prescription. & The European Society of Cardiology 2012. All rights reserved. For permissions please email: journals.permissions@oup.com Vahanian 2012 Eur Heart Journal Expert Valvular Guidelines Relative Contraindication to TAVR Conclusions • BAVs with significant stenosis need to be replaced Repair usually not an option Guidelines and Evidencebased decision-making • Aortopathy should be additionally repaired based on dilation size, pattern and other factors Age General health TAVR Conclusions • Urgent need for bio-markers of aortic progression Biochemical or rheological • Define (in this heterogeneous population) individuals with bicuspid aortopathy in need of aggressive intervention • Guidelines and Evidence-based decision-making Thank you! Question A 45-year old male is referred for surgical evaluation after a 4.6 cm ascending aortic aneurysm was found. Cardiac cath demonstrated no significant coronary artery disease. Echo showed a bicuspid aortic valve with a valve area of 0.8 cm2. Which is true? 1.Aortic pathology is anticipated to show fibrillin 1 2.AVR alone is appropriate 3.A Bentall procedure should be performed if the root is dilated 4.Follow-up of this patient is expected to show an aortic dilatation rate equivalent to patients with tricuspid aortic valves Question 35-year old female with normally functioning BAV, EF 60%, and a root 4.2 cm at the sinus is planning pregnancy 1.Medical management 2.Ascending graft replacement 3.Wheat (AVR+supracoronary ascending) 4.Bio-composite valve graft replacement 5.Valve-sparing root replacement Question A 52-year old man with BAV critical AS and moderate AI. A CT scan reveals a 4.8 cm ascending aortic diameter. Cardiac cath shows no significant coronary artery disease. Each of the following is true EXCEPT: A. If the ascending aorta is not repaired, there is a significant risk of aortic complication within the next 15 years B. A composite valve graft replacement may be needed, especially if the sinuses of Valsalva are dilated C. A separate aortic valve and ascending graft replacement (Wheat operation) may be used if the sinuses of Valsalva are not dilated D. Replacement of the aortic valve alone, and tailoring the native aortic tissue using aortoplasty should stabilize the ascending repair such that graft replacement is unnecessary