CT Imaging of Bicuspid Aortic Valve Disease:

Anatomic patterns favorable and unfavorable for TAVR

and factors influencing device selection

Antonios Chalapas, MD, PhD, FESC

THV & Hygeia Hospital Heart Team

Athens, Greece

Disclosures

I and the HYGEIA Hospital «Heart Team» have received research and travel grants from:

Medtronic, St Jude, Europe, ABBOTT Vascular, Europe

HYGEIA Hospital Heart Team

Cardiologists: A Chalapas, M Chrissoheris, K Papadopoulos, P Kourkoveli, K Spargias

Congenital cardiologist: A. Tzifa

CT Surgeons: N Boumboulis, S Skardoutsos, A Tsolakis, S Pattakos, G Pattakos

Anesthesiologist: I Nikolaou, C. Nastoulis

Vascular Surgeons: I Bellos, S Kaliafas

Radiologists: C Mourmouris, F Laspas

Bicuspid Aortic Valve Incidence Rate

BAV occurring in

15-20% of the TAVR population

not a very rare situation in TAVR population

TAVR in younger population

Bicuspid Aortic Valve Incidence Rate

BAV is the most common congenital valvular abnormality occurring in 1-2% of the general population

Male predominance 3:1

Approximately 10% of 1st-degree relatives have the disorder

Incidence rate is higher in younger patients

Bicuspid Aortic Valve Disease and TAVR

Types of BAV

Morphological heterogeneity

Sievers and Schmidtke Classification

depending on the # of raphes

spatial position of raphes and functional status of the valve

Sievers HH, Schmidtke C. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thorac Cardiovasc Surg. 2007 May;133(5):1226-33.

Circ Cardiovasc Interv. 2019;12:e007107.

Leaflets distribution in the bicuspid cohort

Sizing for TAVR in BAV remains controversial

The aim of the BAVARD (Bicuspid Aortic Valve Anatomy and Relationship With Devices) retrospective multicenter registry is to capture the sizing ratios used for TAVI in BAV and

analyze the 2nd-generation prostheses geometry pos-timplantation.

Circ Cardiovasc Interv. 2019;12:e007107.

Leaflets distribution in the bicuspid cohort

Sizing for TAVR in BAV remains controversial

The aim of the BAVARD (Bicuspid Aortic Valve Anatomy and Relationship With Devices) retrospective multicenter registry is to capture the sizing ratios used for TAVI in BAV and

analyze the 2nd-generation prostheses geometry pos-timplantation.

Representative images of BAV cases

that missed by echocardiography

25-50% of TTE have

non-diagnostic findings

for BAV morphology

due to

severe valvular calcification

type 1 L-R

type 1 L-R

Circ Cardiovasc Interv. 2013;6:284-291

Diagnostic Value of Cardiac CT in Evaluation of BAV

Key Features of BAV Stenosis Patients to be Evaluated for TAVR

ECG-gated cardiac CT

is pivotal for

the accurate morphologic

assessment of BAV stenosis

based on the large variability in BAV

configurations

Circ Cardiovasc Imaging. 2017;10:e005987

Proposed TAVR-Specific BAV Classificationheterogeneity of BAV morphological phenotypes

A. Leaflet morphology is classified on the basis of number of commissures

B. Leaflet orientation is classified on the basis of cusp fusion

A

B

J Am Coll Cardiol Img 2016;9:1145–58

Bicommissural raphe type Bicommissural non raphe typeTricommissural raphe type

Coronary cusp fusion

Coronary cusp fusion

Coronary cusp fusion

Mixed cusp fusion

Mixed cusp fusion

Mixed cusp fusion

Functional” or “acquired” BAV True BAV

23%

56%

21%

Most commonly, the R and L raphe fuse, and the NC sinus is larger than the other sinuses, providing challenges to THV sizing

The aim of the BAVARD (Bicuspid Aortic Valve Anatomy and Relationship With Devices) retrospective multicenter registry is to capture the sizing ratios used for TAVI in BAV and

analyze the 2nd-generation prostheses geometry pos-timplantation.

Circ Cardiovasc Interv. 2019;12:e007107.

TAV BAV

Baseline MDCT analysis

BAV group is characterized by:

• Larger annulus and ascending aorta

• Higher coronaries ostium

• Significantly higher calcium volume

• Ellipticity index was not significantly different

14 centers in the US, Canada, Europe, and Asia, 130 BAV-AS patients underwent TAVR.

Baseline cardiac CT was analyzed by a dedicated Corelab. Outcomes were assessed in line with VARC criteria.

CT Evaluation of BAV Morphology

JACC Img 2016;9:1145–58

Prognostic Implications of Raphe in BAV Anatomy

Association Between Raphe and Significant Valve Dysfunction

JAMA Cardiol. 2017;2(3):285-292

International multicenter registry of 2118 patients with BAVsBw 1991 - 2015

The frequency of significant AR was slightly higher

among pts with BAV with 2 raphes but

evenly distributed among pts with 1 raphe

Significant AS was significantly

higher among pts with BAV with 2 raphes

followed by pts with R-NC cusp fusion

JAMA Cardiol. 2017;2(3):285-292

The presence of a raphe

is associated with increased

rates of all-cause mortality

International multicenter registry of 2118 patients with BAVs

Bw 1991 - 2015

Prognostic Implications of Raphe in BAV Anatomy

Association Between Raphe

and Aortic Dilation and Dissection

JAMA Cardiol. 2017;2(3):285-292

The presence of a raphe

was NOT associated

with the pattern of AORTIC DILATATION

International multicenter registry of 2118 patients with BAVsBw 1991 - 2015

BAV associated Aortopathy

phenotypes

Normal aorta Dilated aortic root Dilated the entire

ascending aorta

and the transverse

aortic arch

Dilated the tubular portion

of the ascending aorta

JAMA Cardiol. 2017;2(3):285-292

38.9%

35-40%

32.4%

28.7%

Difference bw TAV and BAV

Review of 17 studies (series and case report)

Nature Reviews-Cardiology. 2014

BAV and Horizontal aorta

Horizontal aorta is defined as an angle of <30° between the plane perpendicular to the aortic annulus plane and a horizontal reference line

May complicate with

an accurate positioning

of the prosthesis during TAVR,

particularly when using

a self-expanding valve

Porcelain aorta is

more frequent in horizontal aortas

The presence of significant calcifications of the

ascending aorta limits the capability to control

the tension on the delivery catheter during the

device release and may lead to implantation

failure

Aortic Angulation

Attenuates Procedural Success

following Self-Expandable But Not Balloon-Expandable TAVR

J Am Coll Cardiol Img 2016;9:964–72

Angle >70 is an exclusion criteria for clinical trials with SE valves

Type 1 BAV with L-R fusion and calcified fusion

Dilated horizontal aorta

77yo male, NYHA III-IV, LVEF 40%, CABG x3, STS 4.6%, Euroscore I 30%

Horizontal aorta Type 1 BAV

33x28mm

Balloon Valvuloplasty

Nucleus 22x40mm (nominal) x1

VT successfully treated with CV (200J)

Deployment of a Core Valve 31mm

Aortography post CV 31mm Deployment

Moderate to severe PVL

Low implantation

Severe AI due to TVL and PVL

Post dilation

Nucleus 28x40mm (x1)

Final Aortography post Balloon Valvuloplasty

ViV with Core Valve 31mm

Next day

Final Aortography

Mild PVL

CT Post-TAVR in BAV

prosthesis inflow levelmid sinus of Valsalva level

Axial CT scan

Extreme eccentricity of stent frame expansion

Severe PVL

Bicommissural

raphe type

calcified raphe

Bicommissural

raphe type

Non-calcified raphe

Bicommissural

Non-raphe type

Circular expansion

Mild PAR

Mild degree of eccentric

Moderate PAR

Circ Cardiovasc Interv. 2019;12:e007107.

Outcomes in Recent Studies of TAVR in

BAV and TAV Aortic Stenosis

JACC Img 2016;9:1145–58

Circ Cardiovasc Interv. 2019;12:e007107.

Sizing for TAVR in BAV remains controversial

Balloon pre-dilatation was more frequently

carried out in TAV patients (51 vs 28%, P<0.01),

with similar rates of post-dilatation in both

groups (18 vs 20%, P=0.77).

TAVI was a more complex procedure in BAV pts

as expressed by a higher need for a 2nd valve.

No coronary obstruction was observed in TAV or

BAV patients.

Coarctation of aorta may coexist with BAV

• Incidence 35%

• May indispensably eliminate

patient from TAVI and impose

surgical intervention.

• Type 1 L-R may be connected to

aortic coarctation, which is usually

diagnosed in younger age

Current Problems in Cardiology 30 (2005) 470–522.

Type 0 BAV and Coarctation of Aorta

73 year-old female, LVEF 50%, CABG

LE 23.7%, STS mortality 3.2%

Type 0 BAV

Type 0 BAV and Coarctation of Aorta

73 year-old female, LVEF 50%, CABG

LE 23.7%, STS mortality 3.2%

Min. Ø 23.2 mmMax. Ø 30.0 mmAvg. Ø 26.6 mm

Area derived Ø 26.5 mmPerimeter derived Ø 26.9 mm

Area 552.1 mm²Perimeter 84.5 mm

< 48 oAortographyType 0 BAV

Balloon Valvuloplasty

Edwards 23x40mm (x2)

Complete sealing with the 23 Balloon

The diameter of Balloon corresponds to the

small aortic annulus dimension

No AI

Undersize - Sapien 3 26mm

Although in Type 0 BAV the data suggest

the annulus based sizing

from BAV we decided to proceed with undersizing 26

TTE post TAVR

parasternal long axis

Sizing strategies

Annulus-based sizing Sizing based on ICD

Balloon based sizing

Annulus-based sizing

Circ Cardiovasc Interv. 2019;12:e007107.

Device – annulus ratiodegree of oversizing

Sapien 3

Evolut R

Oversizing was applied in both groups,

but lesser in BAV patients

Type 1 BAV with L-R fusion and raphe calcification

Type 0 Aorta

76 year-old male, NYHA III, LVEF 55%, sternotomy due to oesophagous-ectomy

LE 7%, STS mortality 0.9%

Balloon sizing

Min. Ø 20.5 mm

Max. Ø 28.0 mm

Avg. Ø 24.4 mm

Area derived Ø 24.2 mm

Perimeter derived Ø 24.9 mm

Area 461 mm²

Perimeter 78.3 mm

Electrocardiography (ECG)-gated MDCT

Balloon Valvuloplasty

Edwards 20x40mm (nominal)

EV Initially S3

Due to BAV instability (x3)

Evolut R 29mm

1y FU

Valve Sizing for BAV

Balloon sizing

Circ Cardiovasc Interv. 2019;12:e007107.

52%52.5% 14%34%

Sizing based on ICD

Various MSCT measurements at the level of the aortic root

Device – ICD ratio

Sizing ratios utilized for different TAVI devices

in bicuspid cohort

Circ Cardiovasc Interv. 2019;12:e007107.

Sapien 3

Evolut R

No Oversizing

Valve sizing for BAV

Circ Cardiovasc Interv. 2019;12:e007107.

Geometry of THV as Assessed in Post-TAVR MDCT

In BAV pts

Prostheses were

11% smaller than

the mean annulus

diameter at baseline

Circ Cardiovasc Interv. 2019;12:e007107.

Sizing for TAVR in BAV remains controversial

The aim of the BAVARD (Bicuspid Aortic Valve Anatomy and Relationship With Devices) retrospective multicenter registry is to capture the sizing ratios used for TAVI in BAV and

analyze the 2nd-generation prostheses geometry pos-timplantation.

Circ Cardiovasc Interv. 2019;12:e007107.

Prostheses retained

the cylindrical configuration

in BAV and TAV patients

with stable diameters

from their distal edge

to 12 mm above

Post-TAVI MDCT Data

Yoon, SH. et al. J ACC. 2017;69(21):2579–89.

Outcomes of 561 pts with BAV AS and 4,546 pts with TAV AS

compared after PSM, assembling 546 pairs of pts with similar

baseline characteristics.

Procedural and clinical outcomes were recorded

according to VARC-2 criteria.

Conclusion

• BAV is characterized by anatomical heterogeneity

• MSCT sizing - Various MSCT measurements at the level of the aortic root

• Pre-dilatation is mandatory and balloon sizing often is helpful

• BAV with horizontal aorta → Balloon expandable valves

• BAV and Dilated aorta very fragile → Increased risk of Dissection

• Aim at high implantation for leaflet sealing