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Miocardiopatia Hipertrófica
Valor del ecocardiograma
Sociedad de Cardiología de Tucumán
26 de Octubre 2006
Dr. Enrique Alonso
Miocardiopatia Hipertrófica
Valor del ecocardiograma
•
Diagnóstico
•
Pronóstico
•
Tratamiento
Hypertrophic Cardiomyopathy
Left ventricular hypertrophy, symmetric or
asymmetric, in absence of other heart or
systemic disease, which can provoke left
ventricular hypertrophy
Echocardiography for Diagnosis
• Pattern and extent of hypertrophy
• Presence and degree of left ventricular
outflow tract obstruction
• Systolic anterior motion (SAM) of the
mitral apparatus
Hypertrophy:
May involve any and all portions of the left and right ventricle in the small
ventricular cavity and normal left ventricular systolic function.Typical
example of asymmetric left ventricular hypertrophy. Septal thickness
(>15mm) at least 1.3-1.5 times the thickness of the posterior wall.
Concentric hypertrophy in < 10% of cases.
Hypertrophic cardiomyopathy type III:
Parasternal short-axis view showing severe
asymmetric hypertrophy in septal and lateral
segments.
Left Ventricular Outflow Tract Obstruction
Multifactorial mechanisms:
narrowing of outflow tract
anterior displacement of mitral apparatus
hyperdynamic systolic function
Venturi-like effect to pull the mitral valve apparatus into the
left ventricular outflow.
Systolic Anterior Motion of Mitral Valve Apparatus
The M-mode permits a more precise assessment of the
different degrees of mitral valve anterior systolic
movement (arrows).
The magnitude and duration of SAM are related to the
outflow obstruction systolic anterior motion. It is not
pathognomonic of HCM. Hypercontractile states may
buckle the chordae tendinae rather than the mitral valve
leaflet
Parasternal Long-Axis View
M-mode showing SAM and
asymmetric hypertrophy
(septum /PW 1.8)
Anterior systolic movement of
mitral valve.
Longitudinal parasternal view
showing the anterior systolic
movement of the mitral valve in
contact with the interventricular
septum.
Doppler Technique is Fundamental:
•
to assess the presence of left ventricular and / or mesoventricular outflow tract
dynamic obstruction
• to quantify mitral insufficiency
• to rule out fixed aortic valvular or subvalvular stenosis
Representative Diagram of Different Flows in Obstruction Hypertrophic
Cardiomyopathy:
• dynamic obstruction flow in left ventricular outflow tract with predominantly
systolic increased velocity
• mitral insufficiency flow
• normal velocity aortic valve flow with rapid acceleration and meso-telesystolic
deceleration.
•
A displacement of the leaflets may also lead to relative non-coaptation and produce
mitral regurgitation. Typically, the jet of mitral regurgitation is directed laterally or
posteriorly away from the SAM. In a non-posterior jet of MR, independent intrinsic
mitral valve disease should be considered.
Dynamic Subvalvular Gradient
Continuous Doppler from apical view of LVOT with
maximum velocity in telesystole of 6 m/sec, which
permits the calculation of a maximum dynamic
gradient of 152 mmHg.
Dynamic Subvalvular Gradient
When signs of dynamic obstruction are not evident in basal conditions, it
is possible on occasions to provoke them (hypertrophic
myocardiopathies with latent or provokable obstruction) by
manoeuvres that accentuate the pressure gradient:
• amyl nitrite inhalation
• physical exercise
• Valsalva manoeuvre
• isoprenalin perfusion
Indice de desaceleración medio septal- TDI
Relacion con HCM obstructiva:
 IDMseptal, asociado al 58% con obstrucción al TSVI
Asociado a gradientes de 30 mmHg – sensibilidad 93 %
especifidad 91 %
Util en imágenes dificultosas ( test Ejercicio) y en
insuficiencia mitral
Breithardt y Col Heart 2005; 91 : 379-380
Figure 1 ECG invasively measured left ventricular outflow tract (LVOT)
pressure gradient and the tissue Doppler imaging (TDI) velocity trace from
the basal septum. Note the simultaneous development of the LVOT gradient
(open arrow) and the mid systolic septal deceleration notch (solid arrow).
Adapted from Breithardt and colleagues.3
Assessment of Systolic and Diastolic Left Ventricular
Function
Systolic function is normal or hyperdynamic. However,
tissue Doppler assessment shows an abnormal
longitudinal shortening in established HCM or family
members with similar genetic defects. Increasing
muscle mass and abnormal fibrosis provoke left
ventricular stiffness and variable assessment of
degrees of diastolic dysfunction.
Conventional Doppler indices have not been helpful in
assessing diastolic function. Newer markers like tissue
Doppler of mitral annular motion (early peak = e´)
have proved to be far more discriminating in the
assessment of the left ventricular (LV) filling pressures
in HCM. A high ratio E/e´ is indicative of a high filling
pressure in the left ventricle. Mitral septal systolic
velocities are also decreased.
Función diastolica Regional y su
impacto en la función diastolica global
FLUJO
MITRAL
ECO M COLOR
STRAIN
RATE
K Goto JASEcho Julio 2006 vol 19 I 7
Función Diastolica Regional
 El mecanismo mas importante de disfunción
Diastolica Global en HCM, son las
anormalidades de relajación Regional mas
pronunciada en miocardio hipertrófico
 Asincronia Diastolica
 Índice de velocidad de relajación Diastolica
temprana ( ERS) es mas sensitivo.
K Goto JASEcho julio 2006
Disfunción sistólica en HCM
Marcadores Ecocardiograficos:
 Poco frecuente
 Hipertrofia masiva: (p= 0,04), vulnerable a
remodelación, por isquemia, cambios neurohormonales y
hemodinámicas
 Análisis multivariado DSVI, fue un fuerte predictor
 DSVI tubo valor predictivo +, sensibilidad y
especificidad baja.
R Thaman Heart 2005; 91 – 920 - 925
Función sistólica HCM
Strain 2D Dimensional:
Anormalidades sistólicas, subclínicas son
detectadas precozmente con esta técnica
K Serni y Col JAAC vol 47, Issue 6- ;Marzo 2006
Echo in Management of HCM
Care must be taken to reduce variability in gradient changes after
pharmacotherapy, dual chamber pacing, septal ablation and surgical
myectomy. Myocardial contrast echo can be used to identify the vascular
distribution of individual septal perforating branches of the left anterior
descending artery. This is important for correct alcohol septal ablation.
Observe a dynamic gradient of 92 mmHg.
Valor pronostico del diámetro de AI
Registro Italiano de HCM:
Diámetro de AI > 48 mm, tiene un valor predictivo
independientemente de FA con obstrucción en TSVI,
pronostico de muerte relatado a insuficiencia cardiaca
Sinistri y col – AJ of Cardiology vol 98 –I 7 – 2006 octubre
Incidencia de Muerta Súbita
Hipertrofia masiva
100
60
40
20
< 30
30 – 59
>60
P. Sorafja JASEcho junio 2006 vol 1 I 6
Hipertrofia Masiva
Mortalidad
2, 3 % Anual
Evaluación del Tratamiento
Marcapaso DDD y su impacto en el gradiente TSVI
70 % pac. – intima relación entre modificación del
marcapaso (AVI) y reducción gradiente
Gradiente pico TSVI se redujo en 92% de los pac.
92% pacientes mejora clase funcional
Topilsk y col Am J of Cardiology vol 97, I12- 2004
Muchas Gracias
Dr. Enrique Alonso