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  • Miocardiopatías fetales

    Dra. Magdalena Honorato S

    CERPO

    Centro de Referencia Perinatal Oriente

    Facultad de Medicina, Universidad de Chile

  • Miocardiopatías fetales (MC)

     Enfermedad que afecta el músculo cardíaco que no está relacionada con malformaciones estructurales cardíacas

     Afectación de uno o ambos ventrículos

     Puede llevar a falla cardíaca

     Generalmente sin causa aparente

     Recurrencia

  • Generalidades

     8-11% cardiopatías diagnosticadas in útero

     3% cardiopatías en RN

     1/3 mortalidad

     Se presentan entre las 22-24 semanas

  • Clasificación

     Mocardiopatía dilatada

     Miocardiopatía hipertrófica

     Otras:

     Restrictiva

     No compactada

  • Diagnóstico Ecográfico

     Sospecha diagnóstica

     Aumento global tamaño cardíaco

     Dilatación de cavidades

     Engrosamiento e hiperecogenicidad de paredes vetriculares

     Signos de disfunción sistólica/ diastólica

     Ecocardiograma:

     Fundamental para el diagnóstico

    Morfológico

    Funcional

  • Ecocardio fetal

  • Morfología

     Aumento global tamaño corazón

     Aumento ICT MCD

     Dilatación cavidades cardíacas

     Medición de diámetros ventriculares al final de la sístole y diástole MCD

     Medición de grosor pared ventricular al final de sístole y diástole MCH

     Puede existir hiperecogenicidad de paredes ventriculares

     Fibroelastosis endocárdica

  • Morfología

     MCD

     Disminución contractibilidad: Fracción acortamiento < 28%

     Crecimiento ventricular > 97.5% EG

     No compactada

    Observación de trabeculaciones con penetrancia de vasos en el espesor de la pared

     MCH

     Grosor pared > 97,5% EG

  • Función

     Disfunción sistólica VD o VI

     Fracción de acortamiento < o = 28% ( 20cm/ seg)

     Vena umbilical pulsátil

     Hidrops fetal

  • Diagnosis and Prognosis of Fetal Cardiomyopathies Current Pharmaceutical Design, 2010, Vol. 16, No. 26 2931

    merous prominent trabeculations with deep myocardial recesses [13-15].

    In the HCM the diagnosis is made on the basis of an increased

    parietal thickness above 97.5% of normal standards for gestational age [16,17].

    A hemodynamic evaluation can be performed by Doppler mode; pulsed and Color Doppler examination should always be performed and pulsed Doppler wave forms are analyzed at the level

    of the inferior vena cava, hepatic vein (Fig. 5), ductus venosus and umbilical artery, at the level of the atrioventricular (Fig. 6) and semilunar valves, at the level of the outflow tract of the ventricles

    and at the level of the great vessels. Presence of valves regurgita- tion should be documented (Fig. 6). With the Doppler analysis we can measure the left ventricular

    isovolumic relaxation time (IVRT).

    Fig. (4). Isolated non-compaction of the left ventricle at 28 weeks in gesta-

    tion: the fetal echocardiogram shows the persistence of marked ventricular

    trabeculations and deep intertrabecular recesses.

    Fig. (5). Doppler tracings observed in the diastolic function assessment: a

    fetus with DCM at 26 weeks in gestation with an increased a-wave reversal

    in the hepatic vein.

    Systolic and diastolic fetal cardiac functions have become a part

    of the routine evaluation of the fetal heart. It has been suggested, in

    particular, that diastolic function parameters may provide key prog-

    nostic information in several fetal conditions, including cardiomy-

    opathies [18].

    Fig. (6). Doppler tracings observed in the diastolic function assessment: a

    fetus with INVM at 28 weeks in gestation. Fetal Doppler demonstrates

    abnormal E/A ratio (E>A) and mitral valve regurgitation.

    Left and right ventricular systolic function is evaluated by cal-

    culating the shortening fraction (SF). Systolic dysfunction of the left

    or right ventricle is diagnosed when the SF was 28% (< 2 standard deviations (SD) below the mean for previously published normal

    data) [18]. Diastolic dysfunction is proposed [6] when at least two of the following parameters were

    identified: abnormal E/A ratio

    through mitral (Fig. 6) or tricuspid valve inflow (2 SD above the mean for gestational age based

    on the published data[19], increased a-wave reversal in the inferior

    vena cava or hepatic vein (>20 cm/s) (Fig. 5) or a biphasic rather

    than triphasic flow pattern, and the presence of umbilical venous

    pulsations. In the absence of sinus rhythm, the presence or absence

    of umbilical venous pulsations is the only Doppler parameter used

    to evaluate diastolic function. Fetal hydrops

    is diagnosed of a mod-

    erate degree when three sites are identified (hydropericardium,

    ascites, hydrothorax). Hydrops is mild when at least two sites of

    fluid collections were identified and severe with associated skull

    edema.

    DILATED CARDIOMYOPATHY

    Dilated cardiomyopathy is a very rare disease in fetuses. Only isolated case reports and small case series have been reported. The

    published study suggests a very poor outcome for affected fetuses.

    We define an adult patient affected by DCM when he has dila- tation of the cardiac chambers and systolic dysfunction, and also the fetuses with systolic univentricular or biventricular dysfunction as

    affected by dilated cardiomyopathy, with or without chambers di- latations and without increased wall thickness.

    Schmidt et al. [20] in 1986 established the diagnosis of DCM in 6 out of 625 fetuses studied by echocardiography. In all fetuses cardiac structural abnormalities were excluded. Abnormal findings

    included reduced fractional shortening index in 5 patients, 3 were affected by atrioventricular valve regurgitation, 3 with abnormal chamber dimensions and 4 with non-immune hydrops. In 2 fetuses

    with a positive family history of dialated cardiomyopathy, echocar- diographical abnormalities were absent on a first examination per-

    formed during 20 weeks of gestation, but they were present at 30

    weeks of gestation. This suggested that a normal fetal echocardio- gram in a midtrimester fetus does not always rule out the subse- quent development of dilated cardiomyopathy. About the natural

    history, only 2 infants (33%) survived, 1 of whom required heart transplantation during infancy. Death from cardiac failure occurred in 1 fetus and 3 newborns.

  • Compromiso de la función

     Sistólica:

     MCD casi 100%

     MCH 50%

     Diastólica

     MCD/ H 60%

  • Miocardiopatía Hipertrófica

     Aumento de masa o grosor pared ventricular  Causas

     DM:  hipertrofia septal principalmente  Metabolismo anabólico de la hiperinsulinemia y acumulación de glucógeno

    en el 3º trimestre

     RCIU  STFF  Primarias: genes codifican proteinas contráctiles del sarcómero

     B miosia y proteina C ligada a la miosina

     Asociación a Sd. Noonan y mutaciones ADN mitocondrial  Desconocida

     Buen pronóstico: regresión completa en 6 meses sobre todo sin relación a otras anomalías cardíacas

  • 2930 Current Pharmaceutical Design, 2010, Vol. 16, No. 26 Mongiovì et al.

    vernix after the first trimester of pregnancy, has a weak electrical conductivity. Because the electrical activities of the heart cause

    weak changes of the magnetic field, a non-invasive technique, the fetal magnetocardiogram, based on the measurement of the mag-

    netic fields produced in association with cardiac electrical activity,

    provides better signal quality than ECG because of more favorable transmission properties of magnetic signal. This technique has

    higher resolutions in time domain than echocardiography. There are

    many limitations due to significant costs, employment of trained personnel, requirement of a magnetically shielded room, and im-

    mobility of the fetus during the exam. For these reasons, fetal mag-

    netocardiography does not provide a real solution to fetal ECG analysis but it can be helpful in selected conditions. In fact it is the

    best available method to diagnose LQTS, by acquiring an accurate

    averaged signal of the PQRST Complex. In another recent study [4], magnetocardiography was used to better define fetal T-wave

    characteristics including the QT interval in the normal fetus, and to characterize T-wave with the assessment of QT interval and T-wave alternans abnormalities in the fetus with arrhythmia.

    For these reasons and with published studies we will describe

    the fetal CM starting from their echocardiographic presentation:

    dilated cardiomyopathy (DCM) with dilatation of either or both ventricles, and impaired ventricular function (Figs. 1-2), and hyper- trophic cardiom