Bicúspide y dilatación de aorta La imagen aclara el misterio€¦ · Multivariate analysis of...
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Bicúspide y dilatación de aorta La imagen aclara el misterio
Vall d’HebronHospital General UniversitariServei de CardiologiaBarcelona
Arturo Evangelista
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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
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Embriologic hypothesis
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“convexity”
Cotrufo et al. JTCVS; 2005
Della Corte et al. JTCVS; 2008
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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
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BAV Morphotype
73%
24%
18%
L -R
R - N
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0
10
20
30
40
50
60
Normofunctional Aortic Regurgitation Aortic Stenosis
BAVValvular Dysfunction
Valvular prolapse 13% Valvular calcification 25%
%
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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
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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
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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 %
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Ascending Aorta Dilation and BAV
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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%)
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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
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BAV morphotype and aorta diameter by age
LC-RCRC-NC
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%
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
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Aorta dilation phenotype related toBAV type and valvular dysfunction
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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
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J Am Coll Cardiol 2015;66:892–900
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V
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BAV and WSS asymmetry. 4D-flow MRI
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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).
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Válvula Aórtica Bicúspide sin Rafe (Tipo 0)
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Válvula Aórtica BicúspideAsimetría de las sigmoideas
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§ 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.
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s
Circulation. 2019;139:2707–270944% FDR with aorta dilation
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Válvula Aórtica Bicúspide
Eco TC CRM
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- 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
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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
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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%
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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
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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
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13/11/2019VASCERN XXX meeting 35
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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.
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1513
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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