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2012 cardiopatii congenitale

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    CARDIOPATIICONGENITALE

    Prof. Dr. Maria Dorobantu

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    UNTURILE

    Definiie:comunicri anormale ntre cavitiomologe (ASAD, VSVD) sau ntre cavitidiferite (AoAS)

    presiunea de injecie: mare (VS VD)

    mic (AS AD)

    consecina:inegalitatea debitelor ntre circulaiasistemic i cea pulmonar

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    Fiziopatologia untului stg.dr. creterea cronic a Vbal VD (suprasarcin de volum)ncrcare de volum a circulaiei pulmonare HTPde debit(P, R pulmonar N/)

    persitena modificare a vaselor arteriale pulmonareHTP de rezisten !!! inversarea untului=

    sindrom Eisenmenger

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    Clasificare (I)

    Cardiopatii cu unt stg. dr.(necianogene):

    DSA

    DSV

    vene pulmonare aberante

    pesistena de canal arterial (PCA)

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    Sindrom

    Eisenmenger

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    DSA

    Definiie:comunicare interatrial la nivelul septuluiinteratrial

    Tipuri: sinus venos (vene pulmonare aberante)

    ostium secundum

    ostium primum (DSV, cleft VM) foramen ovale patent

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    Sinus venos

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    Ostium secundum

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    Ostium primum

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    Fiziopatologie

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    Clinica Simptomen funcie de dimensiunile untului:

    unt mic

    asimptomatic unt mare :

    pneumonii recurente

    fatigabilitate

    dispnee de efort

    aritmii atriale

    Semne: HAD, HVD

    cianoz de tip central

    PVM auscultaia:

    dedublare fix de Z2 n focarul P

    suflu sistolic pulmonar

    semne de ICD (cnd apare HTP)

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    Paraclinic

    EKG:

    HVD, HAD BRD

    AQRSla dr.

    Rx. toracic: dilataii (AD, VD, a.P.) hipervascularizaie/hipovascularizaie (HTP de

    debit/rezisten)

    Ecocardiografia: dilatare cavitai drepte

    HTP

    micare paradoxal a siv

    vizualizarea defectului

    direcia untului

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    Rx.pulmonar

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    Ecocardiografia 2D

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    Ecocardiografie 2D cu Doppler

    color

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    Ecocardiografia transesofagian

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    Ecocardiografia TE cu Doppler

    color

    di fi f i

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    Ecocardiografia transesofagian cucontrast

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    Complicaii

    HTP

    ICD

    aritmii supraventriculare

    AIT/AVC

    Tratament

    chirurgical/intervenional nchiderea untului (dac DVD>1,5-2 DVS

    sd. Eisenmenger = transplant cord plmn

    medical: se adreseaz complicaiilor

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    DSV

    Definiie: comunicare ntre VS i VD

    Localizare:

    sept membranos

    sept muscular

    asociat sau nu cu alte malformaii

    transpoziie de vase mari

    stenoza P

    DSA

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    Localizare

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    Fiziopatologie

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    Clinica

    Simptome:

    dispnee la efort

    fatigabilitate

    Semne:

    suflul sistolic

    rulment diastolic mitral de debit

    cardiomegalie

    hiperpulsatilitatea VS

    semne de HTP

    cianoz

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    Auscultaia

    suflu pansistolic asociat

    unui DSV mic

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    Auscultaia

    suflu protosistolic n cazde DVS mic intramuscular

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    Auscultaia

    DSV mediu-mare cu unt stg.-dr. mare

    A l i

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    Auscultaia

    DSV larg cu HTP sever

    P li i

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    Paraclinic EKG:

    HAS

    HVS

    HVD

    ax la dreapta

    Rx. toracic: cardiomegalie global

    hiper/hipovascularizaie

    dilatarea a.P.

    Ecocardiografia = diagnostic: evidenierea leziunii

    aprecierea severitii

    complicaii

    Cateterism

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    EKGDSV mic

    DSV larg

    DSV larg

    Eisenmenger

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    Rx. toracic

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    Ecocardiografia 2D cu Doppler

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    Ecocardiografia 2D cu Doppler

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    Ecocardiografia 2D cu Doppler

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    Ecocardiografia 2D cu Doppler

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    Complicaii

    IC

    HTP

    EI

    Tratament

    Chirurgical

    Medical:

    profilaxia EI

    tratamentul IC

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    Fiziopatologia untului dr.stg.

    perfuzarea cordului stng i a circulaiei sistemice cusnge venos:

    cianoz de tip central (intratabil cu O2)

    poliglobulie: tromboze vasculare

    crize anoxice

    dispnee la efort

    debitului n circulaia pulmonar

    ICD

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    Clasificare (II)

    Cardiopatii cu unt dr. stg.(cianogene): canal atrioventricular comun

    maladia Ebstein

    fistule arteriovenoase pulmonare Combinaii unt cu obstacol:

    tetralogia Fallot

    trilogia Fallot

    Anomalii ale circulaiei coronare

    Malpoziii structurale cardiovasculare

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    TETRALOGIA FALLOT

    Definiie: maladie complex ce rezult dinasocierea: DSV nalt

    stenoz subvalvular (infundibular) a a.P.

    aorta ,,clare pe siv

    HVD

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    Fiziopatologie

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    Clinica cianoz

    crizele anoxice

    deficit de dezvoltare

    tromboze

    ICD

    P li i

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    Paraclinic

    EKG: HAD+HVD

    Rx. pulmonar: cord n sabot

    hipovascularizaie

    Ecocardiografia: evidenierea malformaiilor

    aprecierea severitii

    Cateterismul cardiac=obligatoriu

    EKG

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    EKG

    Rx toracic

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    Rx. toracic

    E di fi 2D

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    Ecocardiografia 2D

    E di fi 2D

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    Ecocardiografia 2D

    E di fi 2D

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    Ecocardiografia 2D

    C i i fi

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    Cateterism cu angiografie

    Complicaii

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    Complicaii AVC

    tromboze

    embolii paradoxale

    abcese cerebrale

    hemoragii

    Tulburri ale EAB (acidoz)

    EI

    ICD

    tulburri de dezvoltare

    Tratament chirurgical

    medical (paleativ): betablocante, O2,

    antibiotice

    ANOMALIA EBSTEIN

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    ANOMALIA EBSTEIN

    Definiie:anomalie a valvei tricuspide DSA/DSV membranos/canal atriventricular

    comun:

    tricuspida:

    mult mai jos implantat (atrializarea VD):

    VD mic

    insuficien tricuspidian sever

    IVD

    atrofia foiei septale (practic devine bicuspid)

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    Fiziopatologie

    sngele din AD nu

    se poate vrsa nVD mic

    ptrunde n AS

    prin DSA i apoi n

    circulaia

    sistemic

    Cli i

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    Clinica

    cianoz de la natere

    tipul fr defect septal este neviabil i necesitrealizarea unei comunicri interatriale de la

    natere (intervenional)

    relativ rapid ICD

    risc mare de EI

    frecvent asociat cu WPW

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    Tratament

    Chirurgical:

    reconstrucie valvular

    proteza (de excepie)

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    TRANSPOZITIE CORECTATA

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    TRANSPOZITIE CORECTATA

    DE VASE MARI


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