The Bicuspid AV Surgical Considerations

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The Bicuspid AV Surgical Considerations Ehud Raanani, MD Cardiothoracic Surgery, Sheba Medical Center Sackler School of Medicine, Tel Aviv University September 12, 2014 Homburg

BAV Repair Congenital variations Dysfunction mechanisms, aortopathy Decision making is complex borderline diameters, to root or not? cusps with moderate calcifications Surgical techniques: Stabilization of the root is important annuloplasty is crucial SCA is not a good solution for annular reduction in the BAV there is no standard technique yet

BAV Prevalence 1 2 % Fusion left-right 86 % right-non 12 % left-non 3 % Associated with: Coarctation PDA Turner s syndrome VSD Supravalvar AS William s syndrome Shone s syndrome

Different angles (120-180 degrees)

Freedom from reoperation BAV repair depending on the orientation of the 2 normal commissures Aicher D et al. Circulation 2011;123:178-185

Perfect BAV <1%

Unicuspid Aortic Valve unicuspid bicuspid R. Anderson

Unicuspid AV Eccentric Opening

Mechanisms of AR in BAV Asc. Aortic aneurysm (STJ) Root aneurysm: STJ Annular dilataion Cusp Prolapse Calcific degeneration Root pathology Cusp pathology

BAV Cusps Pathology Cusp Prolapse Raphe fibrosis and caification Fenestration

Cusp Prolapse

Fibrotic and Redundant Raphe

Tissue Deficiency (geometric height< 18-20mm)

Calcified Raphe Pericardial Patch Partial Cusp Replacement

Freedom from reoperation after BAV repair depending on the use of a pericardial patch Other materials(cor-matrix, Gortex membrane, Cardiocell) Aicher D et al. Circulation 2011;123:178-185

Commissural Pathologies

Bicuspid Aortopathy Prevalence of dilatation 20-80% All aorta segments are larger than in TAV Begins in childhood and is progressive Attributed to hemodynamic and genetic: Autosomal dominant, x-linked, familial modes of inheritances were reported Prevalent in first degree relatives Appears also in normally functioning AV

Histology (cystic medial necrosis) Abnormal processing of ECM fibrillin 1 Detachment of smooth muscle layer from ECM Release of MMP s and tissue inhibitors Disruption of matrix and elastin and all media layer

Patterns of Bicuspid Aortopathy AS, male, age>50 R-N pattern AI, male, <40 y genetic

Abnormal trans-valvular flow pattern: (turbulent flow) TAV BAV no. 1 without raphe Flow velocit magnitu [m/s] BAV no. 1 with raphe BAV no. 2

MRI asymmetric flow jet direction

Age sex morphologic type Patho-physiology (AS, AI)

BAV Aortopathy AHA guidelines

BAV Aortopathy

Risk of late aortic events after an isolated aortic valve replacement for bicuspid aortic valve stenosis with concomitant ascending aortic dilation 153 pts, 40-50mm, 1995-2000, 11.3 mean FU years Ascending aortic surgery was required in five patients (3%) Freedom from aortic interventions at 10 and 15 years was 97 and 94%, respectively. No documented aortic dissection or rupture occurred Pts presenting with AI, freedom from adverse aortic events was significantly lower (88 and 70% 10, 15 years) Girdauskas E..Borger MA et al, Eur J cardiothoracic surgery Nov 2012

Dysfunction of Aortic Root Causing AR Sinu-tubular Dilatation Sinu-tubular +/or Annular Dilatation

To Root or Not to Root

Freedom from reoperation after BAV repair in patients with preoperative AVD of >28 mm depending on the use of root replacement. Significant failure in patients with a > 28 mm Annulus whether they have a SCA or Remodeling Root. Aicher D et al. Circulation 2011;123:178-185

Freedom from reoperation after BAV repair depending on operative technique. Root stabilization is important Aicher D et al. Circulation 2011;123:178-185

Ascending Aorta Replacement

Root Remodeling (Yaacoub)

Remodeling and Correction of Dilated Annulus (D3, Lansac)

Re-Implantation (David)

Reimplantation BAV

What are normal annular diameters? Roman 1987 Kim 1996 Nistri 1999 Varnous 2003 Maselli 2005 Babaee 2007 Tamas 2007 Soncini 2009 Bierbach 2010 Zhu 2011 N 135 110 70 100 50 128 32 52 100 315 1132 Annular Ø STJ Ø STJ/ annulus 24.5 (± 3) 27.5 (± 3) 23.4 (± 2.4) 28.1 (± 3.2) 22.7 (± 2.7) 24.7 (± 2.8) 20.55 (± 3) 31.2 (± 3.7) 24.4 (± 4.1) 22.3±1,4 (20.5-32.4) 25.4 (± 4.1) 26.7±2.2 (31.2-23.4) 1.2±0.1 (1.1-1.3) 21.8±2.4 21± 3 21,6 21±2,8 20,3±8,7 29.5±3.1 27± 4 27,3 25± 3,7 23.4±3,1 1.12 1.2 1.1 1.3 1.1 1.3 1.3 1,3 1,2 1,1 Courtesy E Lansac

BAV have dilated AV junction:25-30mm

Courtesy A. Hamdan

Dilated Aortic Annulus Is Very Common in Patients with BAV and AI Is it Important?

Freedom from reoperation after BAV repair depending on preoperative AVD. Aicher D et al. Circulation 2011;123:178-185

Effect of annulus diameter Six geometries with different annulus diameters Calculated by expanding or shrinking the AA of normal case (24mm) The other dimensions were not changed 20mm 22mm 24mm 26mm 28mm 30mm C-C section

Influence of the geometry on coaptation 5 4 average h c [mm] 3 2 1 h C average h C [mm] 0 15 16 17 18 19 geometric height [mm] 3,5 3 2,5 2 1,5 1 0,5 0 20 22 24 26 28 30 AA diameter [mm]

Influence of the geometry on the max. principal stress The average dimensions case (h G =16.2mm, d AA =24mm) σ max [kpa] has the lowest mechanical stress 1000 900 800 700 600 500 400 300 200 100 0 15 16 17 18 19 geometrial height [mm] σ max [kpa] 900 800 700 600 500 400 300 200 100 0 20 22 24 26 28 30 AA diameter [mm] Maximum principal stress [kpa]

Coaptation vs. effective height Comparison of coaptation during diastole as a function of the effective height The effective height correlates well with valve coaptation The cusps in all the cases with h E <9mm prolapsed during 5 diastole h E h c [mm] 4,5 4 3,5 3 2,5 2 1,5 1 0,5 0 7 9 11 13 h E [mm] daa cusp area

Sub-Commissural Annuloplasty

BAV repair (SCA) with dilated annulus (> 27mm): fails in short term SAME Conclusion: More Failure in Large Annulus p = 0.0003 5 years 34 ± 12% 94 ± 5% 86 ± 10% J. Bavaria et al: STS 2013

Sub-Commissural Annuloplasy (SCA) vs Reimplantation on AI p =0.03 SCA 5 years 92 ± 6% 62 ± 10% Bavaria et al; Presented at STS 2013;

Computer Finite Element Model Stress during peak systole TAV has the largest opening area Highest stress values are found in BAVs with fused cusps Raphe region increases stress magnitudes Max. principal stress [kpa] A TAV BAV no. 2 A BAV no.1 without raphe BAV no.1 with raphe

Flow shear stress during peak systole Higher systolic flow shear stresses are found on the cusps of BAVs The TAV model has the lowest shear stress, specifically on the coapting regions Flow shear stress [Pa] TAV BAV no. 2 BAV no.1 without raphe BAV no.1 with raphe

56

Techniques for Aortic Annuloplasty Carpentier 1983 Frater 1986 Haydar 1997 Izumoto 2002 Hahm 2006 Lansac 2007 Schäfers 2009 Fattouch 2011 Courtesy E Lansac

Lansac 2007 Schäfers 2009

Summary BAV repair for AI, with or without Root Aneurysm, is a good operation in selected patients However, there are relative contraindications to repair where the results are sub-optimal: Significant calcification Need for patch augmentation Inter-commissural angle<160 degrees The case of the Large Annulus (>28mm) specially in Normal Root Diameter Aorta dilatation is frequent and usually involves annular dilatation Annular dilatation should be addressed but SCA is probably not the best technique

Thank you

Ascending Aorta Plication for Moderate Dilatation(40-45mm)

Physiological and standardized approach to aortic valve repair + = Remodeling Reimplantation Remodeling + subvalvular annuloplasty

Valsalva 45 mm Valsalva<40 mm all Ø < 40 mm Remodeling + subvalvular annuloplasty Supra-coronary graft + subvalvular annuloplasty (annulus > 25 mm) Subvalvular annuloplasty (annulus> 25 mm)

Frequency of BAV in AVR pts TAV BAV UAV 45 % 49 % 5 %

70% 45% 33% 45% of patients between 71-80 years 70% of patients between 61-70 years!!

70

Survival After the Bentall Procedure in BAV Survival nearly same as age and sex matched group at 12 years 1 Etz C. D. Ann Thorac Surg 2007;84:1186-94 mount sinai NY

Excellent Aortic Bio-Root replacement outcomes in patients < 60y Desai, annals thorac surg 2011;92 2054-61 U-penn

TAVI Valve-in-Valve Adds More Years to the Index Operation Webb Circ 2010 Bapat JTCVS 2012

Fenestration or Perforation Closure and Stabilisation with pericardium

Aortic root aneurysm Valsalva 45 mm Supracoronary aneurysm Valsalva<40 mm Isolated AI all Ø < 40 mm Remodeling + subvalvular annuloplasty Supra-coronary graft + subvalvular annuloplasty (annulus > 25 mm) Subvalvular annuloplasty (annulus> 25 mm) Courtesy E. Lansac

Freedom from reoperation for SVD All Patients < 60 years

Surgical Solutions Geometry altered by non-pressurized state! Stay sutures

Aortic Annuloplasty

Composite AVR

LVOT and Aortic Root Complex

Co-Location

Remodeling and Correction of Annulus (D3) Dilated

Fibrotic and Redundant Triangular Resection

Aortic Valve Replacement Thromboembolism Anticoagulation/Hemorrhage Structural failure PV endocarditis Incidence of valverelated complications Hammermeister et al, JACC 2000

Prolapse Plication of Cusp Margin

Dilatation of the STJ

Position of His bundle Position of RCA Ostia

Velocity vectors and streamlines TAV BAV no. 1 without raphe Flow velocity magnitude [m/s] BAV no. 1 with raphe BAV no. 2