Andrzej Ochala, MD

Medical University of Silesia, Katowice, Poland

Bicuspid aortic valve

o Most common congenital heart disease in adults (1% - 2%)o Most common congenital heart disease in adults (1% - 2%)

o AS is the most common complication of BAV

o Patophysiologyo Patophysiology

o accelerated leaflet degeneration

o calcificationo calcification

o endothelial dysfunction

o abnormal valve hemodynamics

abnormal flow in aortic rooto abnormal flow in aortic root

o AVR necessary in 20% - 50% patients with BAV

o BAV in ca. 50% of patients undergoing isolated SAVR for ASo BAV in ca. 50% of patients undergoing isolated SAVR for AS

Circulation. 2005;111:920-925Circulation 2002;106:900–4.

J Am Coll Cardiol 2010;55:2789–800.

Bicuspid aortic valve

o Most common congenital heart disease in adults (1% - 2%)o Most common congenital heart disease in adults (1% - 2%)

o AS is the most common complication of BAV

o Patophysiologyo Patophysiology

o accelerated leaflet degeneration

o calcificationo calcification

o endothelial dysfunction

o abnormal valve hemodynamics

Abnormal flow in aortic root

30-45% of TAVI candidates

o Abnormal flow in aortic root

o AVR necessary in 20% - 50% patients with BAV

o BAV in ca. 50% of patients undergoing isolated SAVR for ASo BAV in ca. 50% of patients undergoing isolated SAVR for AS

Circulation. 2005;111:920-925Circulation 2002;106:900–4.

J Am Coll Cardiol 2010;55:2789–800.

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Classification - based on number ofraphesraphes

Type 0No raphe

Type 1One raphe

Type 2Two raphesNo raphe One raphe Two raphes

’Pure BAV’

Sievers & Schmidtke. J Thorac Cardiovasc Surg 2007; 133:1266-33

- based on spatial position of raphe- based on spatial position of raphe

Sievers & Schmidtke. J Thorac Cardiovasc Surg 2007; 133:1266-33Sievers & Schmidtke. J Thorac Cardiovasc Surg 2007; 133:1266-33

7Hasan Jilaihawi et al, In press, JACC Imaging

62,3%62,3%

Patients aged 50-70 years undergoing AVRPatients aged 50-70 years undergoing AVR

16.7 ≥75% of patients with a BAV might have been precluded from undergoing TAVI

Reported experiences derive from a very

Patients aged 50-70 years undergoing AVR

KTW(N=244)

Reported experiences derive from a veryselected subset of patients.

Roberts WC, Ko JM. Frequency of unicuspid, bicuspid and tricuspid aortic valves by decade in adults having aortic valve replacement for isolated aortic stenosis. Circulation 2005;111:920–5.Zhao, Z.G., et al., Transcatheter aortic valve implantation in bicuspid anatomy. Nat Rev Cardiol, 2015. 12(2): p. 123-8.

8Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg 2007;133:1226–33.

Patients with BAV havePatients with BAV have

• >100% larger volume of calcifications• >10% annulus perimeter• a higher risk of valve underexpansion• a higher risk of valve underexpansion• a higher post procedural aortic gradient• a trend toward a higher risk of PVL.

compared to TAV patients.

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compared to TAV patients.

Watanabe, Y., et al., Comparison of multislice computed tomography findings between bicuspid and tricuspid aortic valves before and after transcatheter aortic valve implantation.Catheter Cardiovasc Interv, 2015. 86(2): p. 323-30.

1. Large annulus1. Large annulus

2. Horizontal, dilated aorta

3. Elliptic orifice

4. High calcium burden4. High calcium burden

A. Valve sizing

B. Valve positioningB. Valve positioning

C. Aortic dissection, progression of ascending aorta dilatation

D. Paravalvular leaks

E. Conduction disturbanceE. Conduction disturbance

10Hope MD et al. Bicuspid aortic valve: four-dimensional MR evaluation of ascending aortic systolic flowpatterns. Radiology 2010;255:53–61.

Annulus 27 x 35 mm (perimeter96 mm)

Sinus of Valsalva 44 x 51 mm

ST-J 44 x 44 mm

Ascending aorta 45,1 mm

25 mm balloon sizing Medtronic CoreValve 31

LVOT 24 x 34 mm

LM 15,1 mm

RCA 19,8 mm

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12Girdauskas E, Rouman M, Disha K, et al. Functional aortic root parameters and expression of aortopathy in bicuspid versus tricuspid aortic valve stenosis.J Am Coll Cardiol 2016;67:1786–96.

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15TTE: S: v max 2,5 m/s, P max 25 mmHg, P mean 12 mmHg

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17TTE: S: v max 2,0 m/s, P max 15 mmHg, P mean 8 mmHg

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19

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21TTE: Pmax 38 mmHg, P mean 22 mmHg

VALVE SIZE IN A BICUSPID VALVESVALVE SIZE IN A BICUSPID VALVES

Avoid oversizingAvoid oversizing- risk of rapture

- large self-expanding valves may have less radial- large self-expanding valves may have less radial

force

Usually it is safe to undersizeUsually it is safe to undersize

Very large annuli may still be suitable for TAVIdue to higher degree of calcificationdue to higher degree of calcification

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25First Explantation of Direct Flow Medical Transcatheter Valve. Scholtz et al. J Heart Valve Dis.2015 May;24(3):342-4.

Significant Transaortic Gradient After Direct FlowAortic Valve Implantation. Rosa-Ana Hernandez-Antolin et al. CCI 86:347-352 (2015)

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Perlman GY, Blanke P, Dvir D, et al. Bicuspid aortic valve stenosis: favorable earlyoutcomes with a nextgeneration transcatheter heart valve in a multicenter study. JAm Coll Cardiol Intv 2016;9:817–24.

Procedural Complications

Complications Rate

Valve embolization 0%

Procedural Complications

Valve embolization 0%

Second valve 0%

Annular rupture 0%Annular rupture 0%

Coronary occlusion 0%

Conversion to surgery 0%Conversion to surgery 0%

Post implant gradient >20mmHg 1.9%

G Perlman, Vancouver, TCT 2015

Factors that contribute to suboptimal TAVRFactors that contribute to suboptimal TAVRoutcomes inpatients with BAV are:

Fused raphe with variable degrees of calcificationAsymmetry of aortic valve cuspsAnnular eccentricityAnnular eccentricityCalcification of the left ventricular outflow tract,Aortopathy and aortic root dilation that may

render anchoring the THV problematicrender anchoring the THV problematic

Polish experience with TAVI in BAV

42 patients with bicuspid aortic valve

(confirmed with MSCT)

High risk: LOG ES 19,7 High risk: LOG ES 19,7

Prostheses:

CoreValve 10

Evolut R 6 Evolut R 6

Sapien XT 6

Sapien 3 11

Lotus 9

Device success 93%

Mean pressure gradient 15.6 mmHg

30d mortality: 7% 30d mortality: 7%

30d stroke: 5%

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TAVI in BAV we should remember:

o Supra-annular measurements and high implantationo Supra-annular measurements and high implantation

o Oversizing of the valve should be avoided

o Rupture of the ascending aorta in patients with ascending aneurysm may occuro Rupture of the ascending aorta in patients with ascending aneurysm may occur

o Balloon sizing may be better than sizing based on CT only

o Patients with large annulus may still be suitable for TAVI if the valve is bicuspido Patients with large annulus may still be suitable for TAVI if the valve is bicuspid

o MSCT-guided assessment is a key part of procedural planning especially

in intermediate and lower surgical risk groups who are younger and in

whom BAV is more common

The Bicuspid Aoritc Valve and TAVI- Conclusions

Not an exclusion for TAVI

Associated aoric root dilation may argue for surgery

Larger annuli may require larger THVs

Finding a coplanar view and positioning may be difficult Finding a coplanar view and positioning may be difficult

Pacemaker rates may be increased

PVL rates may be increased

Postprocedural gradient may be increasedPostprocedural gradient may be increased

Second generation TAVI systems may allow for Better sealing of asymmetrical AV cusp orifice

Self-adapting Self-adapting

Sealing dress

Respositioning

Need for Need for stronger clinical evidence (research cooperation)

TAVI system developments to address BAV anatomy31