Bicúspide y dilatación de aorta La imagen aclara el misterio

Vall d’HebronHospital General UniversitariServei de CardiologiaBarcelona

Arturo Evangelista

Ascending aorta dilation increases with age. At 60s, 80% had aorta dilation.

After 25 y of follow-up 40% of patients presented AA aneurysms but only 1% of AD.

Predictors of aneurysm formation: Significant valvular disease (AS or AR)

Initial dimeter > 40mm

JAMA 2011;306:1104-13

Embriologic hypothesis

“convexity”

Cotrufo et al. JTCVS; 2005

Della Corte et al. JTCVS; 2008

Results of the Spanish network on Bicuspid Aortic Valve

H. Vall d´HebrónBarcelona

H. ArrixacaMurcia

H. Clínico de MálagaH. Virgen Macarena

Sevilla

H. 12 de OctubreH. Gregorio Marañón

Madrid

H. Clinico Valladolid

Complexo HospitalarioVigo

852 patientsmean age: 47.4±16.8 years

70.4% male

Heart. 2018 Apr;104(7):566-573

BAV Morphotype

73%

24%

18%

L -R

R - N

0

10

20

30

40

50

60

Normofunctional Aortic Regurgitation Aortic Stenosis

BAVValvular Dysfunction

Valvular prolapse 13% Valvular calcification 25%

%

0

10

20

30

40

50

60

Normofunctional Aortic Regurgitation Aortic Stenosis

Type LC-RC Type RC-NC

p= ns

BAV Valvular Phenotype andValvular Dysfunction

%

p=0.01

56%

48%

25%23%

20%

30%

p=0.05

Risk factors and Aortic Valve DysfunctionNORMO AS AR P

AGE < 50 y 57,7% 13,9% 28,4%

AGE > 50 y 48,9% 29,3% 21,8% ,000

No HTA 56,1% 12,5% 31,4%

HTA 49,8% 21,0% 29,2% ,014

No DYSLIPIDAEMIA 56,7% 11,1% 32,2%

DYSLIPIDAEMIA 44,3% 28,1% 27,5% ,000

No DIABETES 54,2% 13,8% 32,0%

DIABETES 47,4% 39,5% 23,2% ,000

No SMOKERS 58,5% 11,1% 30,5%

SMOKERS 44,7% 23,2% 32,0% ,000

Age and Valvular Dysfunction in BAV

0

5

10

15

20

25

30

35

40

<= 30 años (N:138) 31-50 (N:271) 51-70 (N:265) >= 70 (N:52)

5,8

18,1

27,5

38,5

33,3

25,8

23,8

11,5

AS %

AR %

Ascending Aorta Dilation and BAV

BAV and Aortic Dilation

Aortic dilation was diagnosed in 663 patients (79.6%).

Aortic root in 282 patients (33.9%) and ascending aorta in 632 (75.9%)

0

5

10

15

20

25

30

35

40

45

Root Tubular Arch

Type LC-RC Type RC-NC

BAV Valvular Phenotype andAorta Diameters

mm

37.5 ± 5.6

34.3 ± 5.3

41.2 ± 7.639.9 ± 7.4

30.9 ± 5.1

33.3 ± 6.3

p=0.001 p=0.001p:ns

BAV morphotype and aorta diameter by age

LC-RCRC-NC

%

0

10

20

30

40

50

60

NORMO-FUNCTION AR AS

26

18 16

27

41

23

47

41

60

No dilatation

Aortic root dilatation

Tubular dilatation

Relación entre la disfunción valvular y la dilatación de aorta

Evangelista et al. Heart. 2018 Apr;104(7):566

Aorta dilation phenotype related toBAV type and valvular dysfunction

Multivariate analysis of aorta dilation phenotype

Aortic root phenotype Odds ratio CI 95 % P-value

Age 0.98 0.97-0.99 0.005

Male 2.95 1.64-5.29 0.001

Valve morphotype II 0.27 0.13-0.53 0.001

Valve morphotype III 2.52 0.96-6.60 0.060

AS-dominant, n(%) 0.27 0.12-0.62 0.002

AR-dominant, n(%) 1.57 1.01-2.44 0.044

Ascending aorta phenotypeAge 1.01 1.00-1.02 0.002

Male 0.54 0.38-0.78 0.001

Valve morphotype II 1.08 0.75-1.56 0.648

Valve morphotype III 0.44 0.19-1.04 0.062

AS-dominant, n(%) 2.44 1.59-3.77 0.001

AR-dominant, n(%) 1.33 0.92-1.90 0.122

J Am Coll Cardiol 2015;66:892–900

V

BAV and WSS asymmetry. 4D-flow MRI

44mm

52mm

48mm

44 mm

44mm

Root asymmetry (≥ 5mm) was present in 50% of patients with type 0 BAV and 39% of those with type 1 BAV. In 20 (39%) of patients with BAV-LR (types 0 and 1) and 11 (48%) of those with BAV-RN (types 0 and 1).

Válvula Aórtica Bicúspide sin Rafe (Tipo 0)

Válvula Aórtica BicúspideAsimetría de las sigmoideas

§ 256 families. The recurrence rate of BAV was 6.4% in FDR (9.2% in men, 3.5%in women).

§ Aortic dilation was diagnosed in 9.6% of FRD with TAV, with a rootphenotype in 2.7% and tubular in 6.9%.

§ Angio CT in 22 FDR with aortic dilation from the coordinator centre, in 9 ofwhom mini-raphe, not defined by TTE, was diagnosed and 5 underwent 4D-MRI study which showed eccentric jet with an increase in rotational flow andaxial wall share stress.

Heart. 2019 Oct 15.

s

Circulation. 2019;139:2707–270944% FDR with aorta dilation

Válvula Aórtica Bicúspide

Eco TC CRM

- Medida de referencia con TC/CRM siexiste dilatación aórtica paracomparaciones fiables en caso deprogresión.

- Comparaciones con la misma técnica deimagen side-by-side.

- Sincronización con ECG en telediástole

- Variabilidad inter e intraobservador paraTC es de 3 mm por lo que se considerasignificativo > 5 mm

- Debe incluirse la pared mas anterior en lamedición de diámetros aórticos. Datospronósticos recientes derivan demediciones que incluyen la pared aórtica

Ilaria Dentamaro, Laura Galian, Rubén Fernández-Galera, Gisela Teixidó, María Teresa González-Alujas,Laura Gutiérrez, Chiara Granato, Nicolás Villalva, F. Calvo, Violeta Sanchez, Josep María Alegret,

Paolo Colonna, Antonella Moreo, Rodolfo Citro, Fabio Chirillo y Arturo Evangelista

7

6

5

42

31

459 pacientes (entre 18-89 años)Edad media: 52,0 ± 17,3 años 70,8% hombres

El seguimiento ecocardiográfico: entre 5-12 años con una mediana de 8,3 ± 3,4 años

RESULTADOSDilatación de aorta ascendente y progresión en la dilatación

• El 83,2% morfotipo tubular (tubular > raíz)

Dilatación (> 45 mm) en el estudio basal en el 39%

RAIZ AORTA ASCENDENTE

7% 32%

Al final del seguimiento raíz > 45 mm: 7%à 16% aorta ascendente > 45 mm: 32%à 41%

P= 0,019

0,33 ± 0,29 mm 0,38 ± 0,31 mm

Dilatación anual de la Ao

Predictores de Dilatación de la Aorta Ascendente

Variables Coeficiente valor p 95% Conf. Intervalo

Edad - 0,014 0,079 -0,003-0,001

Mujer 0,003 0,919 -0,063-0,070

Hipertensión -0,067 0,028 -0,127-0,007

Diabetes -0,056 0,404 -0,189-0,076

Dislipidemia -0,012 0,751 -0,0863-0,062

Tabaquismo 0,044 0,230 -0,028-0,117

VAB-DN versusVAB-DI

0,030 0,402 -0,041-0,102

VAB-IN versus VAB-DI

-0,141 0,391 -0,464-0,182

Rafe -0,056 0,101 -0,124-0,011

EAo significativa 0,111 0,053 -0,001-0,224

IAo significativa 0,005 0,888 -0,073-0,085

Morfotipo raíz 0,132 0,009 0,033-0,231

Asociación entre las características del paciente y el crecimiento anual de la raíz aórtica

RAIZ AORTA ASCENDENTE

Variables Coeficiente valor p 95% Conf, Intervalo

Edad -0,0009 0,340 -0,002- 0,000

Hombre 0,0962 0,010 0,023-0,168

Tabaquismo 0,0681 0,093 -0,011-0,148

Diabetes -0,135 0,080 -0,287 -0,016

Rafe 0,068 0,082 0,008 -0,145

EAo mod-sev 0,14770 0,021 0,021- 0,273

13/11/2019VASCERN XXX meeting 35

Conclusions

§ BAV is intrinsically dysfunctional with excentric jet dirested to the aortic wall. AA is dilated in 70% of normofunctioning AV.

§ BAV type R-L has more aortic root dilation than type R-N; however, type R-Ndilates more frequently in the tubular part and aortic arch.

§ Patients with aortic root dilation phenotype were younger, more likely to bemale, had increased frequency of AR but low frequency of AS and R-N.

§ Ascending aorta dilatation appears depend on:a) Different haemodynamic factors such as increase wall stress secondaryto jet excentricity and valve dysfunction.b) Genetic factors could lead to intrinsic aortic wall weakness.

1513

Results of the Spanish network on Bicuspid Aortic Valve

H. Vall d´HebrónBarcelona

H. ArrixacaMurcia

H. Clínico de MálagaH. Virgen Macarena

Sevilla

H. 12 de OctubreH. Gregorio Marañón

Madrid

H. Clinico Valladolid

Complexo HospitalarioVigo

852 patientsmean age: 47.4±16.8 years

70.4% male

Heart. 2018 Apr;104(7):566-573

GRACIAS