+ All Categories
Home > Documents > Curs Studenti Cardiomiopatii

Curs Studenti Cardiomiopatii

Date post: 30-May-2018
Category:
Upload: ayannapui
View: 232 times
Download: 0 times
Share this document with a friend

of 57

Transcript
  • 8/14/2019 Curs Studenti Cardiomiopatii

    1/59

    Cardiomiopatiile

  • 8/14/2019 Curs Studenti Cardiomiopatii

    2/59

    Definitie

    Afectiune a miocardului asociata cualterarea structurala si functionala amuschiului cardiac in absenta cardiopatieiischemice, a hipertensiunii arteriale, avalvulopatiilor sau bolilor cardiacecongenitale care sa explice afectareamiocardica.

  • 8/14/2019 Curs Studenti Cardiomiopatii

    3/59

    Clasificare

    CMP Familiale: afecteaza mai multi membri ai unei familii CMP non-familiale: afecteaza un singur membru al unei familii idiopatidobandite (in cadrul altor afectiuni)

  • 8/14/2019 Curs Studenti Cardiomiopatii

    4/59

    Cardiomiopatia Dilatativa Idiopatica

  • 8/14/2019 Curs Studenti Cardiomiopatii

    5/59

    CMD - Definitie

    Sindrom caracterizat prin dilatare si disfunctie sistolicaventriculara stanga

    Dilatarea si disfunctia ventriculului drept pot fi

    prezente dar nu sunt obligatorii pentru diagnostic

    In absenta unor conditii anormale de umplere VS (HTA,

    valvulopatii) sau a cardiopatiei ischemice cu disfunctiecontractila VS secundara !

  • 8/14/2019 Curs Studenti Cardiomiopatii

    6/59

    Incidenta

    5-8 cazuri / 100.000 locuitori / an

    De 3X mai frecvent la afro-americani si la sexul masculindecat la caucazieni si respectiv sexul feminin

    CMD simptomatica 10 - 50% mortalitate la 1 an

    Rata anuala a mortalitatii 11-13%

    25% din pacientii cu CMD cu debut recent se pot amelioraspontan

  • 8/14/2019 Curs Studenti Cardiomiopatii

    7/59

    Supravietuirea observata in cadrul unui lotde 104 pacienti

    YearsAm J Cardiol 1981; 47:525

    Cardiomiopatia dilatativa idiopatica

  • 8/14/2019 Curs Studenti Cardiomiopatii

    8/59

    CMD Anatomie patologica

    Dilatarea celor 4 camere cardiace Ventriculi > atrii

    Trombi intracavitari

    Histologie Arii de fibroza interstitiala siperivasculara

    Miocite hipertrofice / atrofiate

    Nu exista markeri imunologici, histochimici, morfologici, ultrastructurasau microbiologici specifici !

  • 8/14/2019 Curs Studenti Cardiomiopatii

    9/59

    CMD Etiologie

    1. Genetica si familiala 2. Virala/Citotoxica 3. Imunologica

    Proteine ale sarcomerului:Lanturi grele de miozinaLanturi usoare de miozinaLanturi reglatoare de miozinaActinaTroponina TTroponina IAlfa-tropomiozinaProteina C care leaga miozina

    Banda ZProteine citoscheletale:DistrofinaDesminaMetavinculina

    Membrana nucleara:

    LamininaEmerina

  • 8/14/2019 Curs Studenti Cardiomiopatii

    10/59

    Tablou clinic

    Debut simptomatic la subiecti de varsta medie(B>F)

    Simptomele apar de obicei progresiv

    Dialatarea ventriculara precede uneori aparitiasimptomelor

    Debut acut dupa un episod infectios viral

    Si t i d IC

  • 8/14/2019 Curs Studenti Cardiomiopatii

    11/59

    Simptome si semne de IC

    Simptome de insuficienta cardiaca:congestie pulmonara

    dispnee (repaus, efort, nocturna), ortopnee

    congestie sistemicagreata, dureri abdominale, nicturie

    debit cardiac scazutfatigabilitate, slabiciune musculara

  • 8/14/2019 Curs Studenti Cardiomiopatii

    12/59

    Examen fizic

    Semne de insuficienta cardiaca

    hipotensiune, tahicardie, tahipnee, turgescenta jugulara hepatomegalie pulsatila edeme periferice ascita embolii sistemice

    AMC crescute in sens transversal

    Impuls apical deplasat lateral

    Zg3, Zg4

    Zg 2 dedublat (HTP) Suflu sistolic de regurgitare mitrala/tricuspidiana

  • 8/14/2019 Curs Studenti Cardiomiopatii

    13/59

    Examinari de laborator

    ECG

    Rx cardio-pulmonara Holter ECG (ameteala, palpitatii, sincope) Ecocardiografie Cateterism cardiac

    F t i l ti i CMD

  • 8/14/2019 Curs Studenti Cardiomiopatii

    14/59

    Factori cu rol prognostic in CMD

    Clinici Neinvazivi Invazivi

    Clasa NYHA III/IV FE scazuta Presiuni de umplere

    Varsta avansata Dilatare VS marcata

    Cons. O2 la efort Masa VS

    ( moderata

    Aritmii ventriculare Disfunctie diastolica

    complexe

    Semne de stimulare Rezerva contractila scazuta

    simpatica excesiva

    Galop protodiastolic Dilatare sau disfunctie de VD

    M t l CMD

  • 8/14/2019 Curs Studenti Cardiomiopatii

    15/59

    Managementul CMD

    Limitarea activitatii in functie de statusul functional

    Restrictie sodata 2-g Na+ (5g NaCl)

    Restriction de fluide in caz de hiponatremii severe

    Tratament medicamentos:

    Betablocante

    ACE inhibitori

    Diuretice de ansa

    Spironolactona

    Combinatii de hidralazina / nitrati

    Digoxin

    Anticoagulant (FE< 30%, trombi intracavitari, istoric de tromboembolism periferic, Fia)

    dopamina, dobutamina si/sau inhibitori de fosfodiesteraza iv

  • 8/14/2019 Curs Studenti Cardiomiopatii

    16/59

    Managementul CMD

    Tratament imunosupresivResincronizareTratament chirurgical (valva mitrala, remodelare VS)

    Dispozitive de asistare ventricularaTransplant cardiac

    Indicatii clinice ale biopsiei

  • 8/14/2019 Curs Studenti Cardiomiopatii

    17/59

    Indicatii clinice ale biopsieiendomiocardice

    1. IC cu debut recent < 2 sapt asociata cu dilatare ventriculara sicompromitere hemodinamica (I)

    2. IC cu debut intre 2 sapt si 3 luni asociata cu dilatare ventricularasi aritmii ventriculare nou aparute, BAV II, III si lipsa de raspuns l

    masurile terapeutice uzuale (I)

  • 8/14/2019 Curs Studenti Cardiomiopatii

    18/59

    Cardiomiopatia hipertrofica (CMH)

    C di i ti hi t fi

  • 8/14/2019 Curs Studenti Cardiomiopatii

    19/59

    Cardiomiopatia hipertrofica

    Prevalenta 0.02 - 0.2%

    Ventricul stang hipertrofiat si nedilatat, in absenta altorcauze de HVS (disfunctie predominant diastolica)

    Cavitate VS mica, HVS asimetrica, miscarea sistolica

    anterioara a valvei mitrale (SAM)

  • 8/14/2019 Curs Studenti Cardiomiopatii

    20/59

    Sept si perete anterolateral VS - frecvent Perete posterobazal - rar

  • 8/14/2019 Curs Studenti Cardiomiopatii

    21/59

    CMH - Histologie

    Hipertrofie miocitara

    Dezorganizare miocitara disarray > 5% din miocard

    CMH Fiziopatologie

  • 8/14/2019 Curs Studenti Cardiomiopatii

    22/59

    CMH - Fiziopatologie

    Sistola

    Gradient dinamic in tractul de golire al VS

    Diastola

    Alterarea umplerii diastolice, presiunii de umplere, dilatare atrialaimportanta

    Ischemie miocardica

    masei musculare, presiunilor de umplere, cons. O2

    rezerva vasodilatorie, densitatea capilara

    Compresie sistolica a coronarelor intramurale

    CMH Familiala

  • 8/14/2019 Curs Studenti Cardiomiopatii

    23/59

    CMH Familiala

    Transmitere autosomal dominanta in 50% din cazuri

    10 gene diferite ale prot. sarcomerice cu peste 150 de mutatii

    Lantui grele de betamiozina

    Lanturi usoare demiozina

    Lanturile reglatoare ale

    miozinei Actina

    Troponina T

    Troponina I

    Alfa-tropomiozina

    Proteina C care leagamiozina

    CMH - Tablou clinic

  • 8/14/2019 Curs Studenti Cardiomiopatii

    24/59

    CMH - Tablou clinic

    Asimptomatic, descoperire ecocardiografica

    MS poate reprezenta prima manifestare

    Simptomatic adulti 40-50 ani

    dispnee - 90%

    angina pectoris - 75%

    fatigbilitate, pre-sincopa, sincoparisc de MS la opii si adolescenti

    palpitatii, DPN, IC rarefortul accentueaza simptomele

    CMH E fi i

  • 8/14/2019 Curs Studenti Cardiomiopatii

    25/59

    CMH - Examen fizic

    Impuls apical sustinut Zg 4 (contractie atriala viguroasa) Unda a proeminenta puls venos jugular Suflu sistolic aspru crescendo- descrescendo careincepe dupa Zg 1 si se ausculta intre apex simarginea stanga a sternului (nu iradiaza pe vaselegatului)

    Manevre care gradientul si suflul

  • 8/14/2019 Curs Studenti Cardiomiopatii

    26/59

    Manevre care gradientul si suflul

    ContractilitatePresarcina Postsarcina

    Valsalva (strain) --- Standing --- --

    Postextrasistolic --

    isoproterenol

    Digitala --

    Nitrit de amil --

    Nitroglicerina ---

    Efort

    Tachicardie --

    Hipovolemie

    Manevre care gradientul si suflul

  • 8/14/2019 Curs Studenti Cardiomiopatii

    27/59

    Manevre care gradientul si suflul

    ContractilitatePresarcina Postsarcina

    Manevra Mueller ---

    Valsalva (overshoot) ---

    Squatting ---

    Ridicarea pasiva a picioarelor --- -

    Fenilefrina --- --

    Beta-blocantele --

    Anestezia generala -- --

    Efortul izometric --- --

    CMH vs Stenoza Aortica

  • 8/14/2019 Curs Studenti Cardiomiopatii

    28/59

    CMH vs Stenoza Aortica

    CMH Obstructie fixapuls carotidian spike and dome parvus et tardus

    suflu radiat pe carotidevalsalva, standing

    squatting, handgrippassive leg elevation

    tril sistolic sp IV ic stang sp II ic drept

    clic sistolic absent prezent

    CMH L b t

  • 8/14/2019 Curs Studenti Cardiomiopatii

    29/59

    CMH - Laborator

    ECG normal 15-25% HVS Corelatie slaba intreHVS pe ECG si la

    ecocardiografie Unde T gigantenegative CMH apical(japonezi)Q DII,III,aVF, V2-6

    Aritmii supraventriculare Fia

    Aritmii ventriculare TVNS, TV

    Rx, ECG, Holter ECG, SEF

    CMH E di fi

  • 8/14/2019 Curs Studenti Cardiomiopatii

    30/59

    CMH - Ecocardiografie

    SIV si/sau PP > 15 mm 60 mm

    VMA lungaproemina inTEVS

  • 8/14/2019 Curs Studenti Cardiomiopatii

    31/59

    Istoria naturala

  • 8/14/2019 Curs Studenti Cardiomiopatii

    32/59

    mortalitate anuala 3% in centrele tertiare,probabil 1% in general

    risc de MS mare la copii 6%/an

    deterioare clinica lenta

    progresie spre CMD in 10-15% din cazuri

    Factori de risc pentru MS

  • 8/14/2019 Curs Studenti Cardiomiopatii

    33/59

    Factori de risc pentru MS

    Istoric de MS (FV) Istoric familial de MS

    TVSIstoric de sincope

    Magnitudinea HVS > 30 mm

    Raspuns TA anormal laefort

    TVNS (Holter)

    FiA

    Ischemia miocardica Obstructie TEVS Mutatii cu risc crescut Sportul competitiv

    Majori Posibili

    Recomandari pentru activitate competitiona

  • 8/14/2019 Curs Studenti Cardiomiopatii

    34/59

    Recomandari pentru activitate competitiona

    Evitarea sporturilor competiotionale indiferentdaca exista sau nu gradient dinamic

    CMH - Management

  • 8/14/2019 Curs Studenti Cardiomiopatii

    35/59

    g

    Betablocante (Metoprolol) prima linieefect inotrop si cronotrop negativ

    scad consumul miocardic de O2

    amelioreaza umplerea diastolica

    reduc severitatea anginei si efectele negative ale obstructiei TEVS

    Blocante de calciu (Verapamil, Diltiazem) in caz deineficienta sau intoleranta a medicatiei BB

    efect inotrop si cronotrop negativ

    amelioreaza umplerea diastolica

    Atentie: Efect vasodilatator impredictibil, precautie in CMHO!

    CMH - Management

  • 8/14/2019 Curs Studenti Cardiomiopatii

    36/59

    Disopiramida agent antiaritmic de clasa IA- alternativa

    la BB sau CCBinotrop negativsuprima aritmiile ventriculareutila in CMHO

    Amiodarona, sotalol

    Miotomie miectomie (procedura Morrow) Ablatia septala prin alcoolizare

    Plicaturarea VMADDD pacing

    CMH - Management

  • 8/14/2019 Curs Studenti Cardiomiopatii

    37/59

    CMH vs cordul atletului

    CMH Atlet

    + Patern particular HVS -+ Cavitatea VS 55 mm ++ Dilatarea AS -+ Patern ECG bizar -+ Anomalii umplere VS - +

    Sex F -- HVS cu deconditionarea ++ Istoric familial de CMH -

    Circulation 1995; 91:1596

  • 8/14/2019 Curs Studenti Cardiomiopatii

    38/59

    Cardiomiopatia restrictiva

  • 8/14/2019 Curs Studenti Cardiomiopatii

    39/59

    Cardiomiopatia restrictiva

    Trasatura definitorie: disfunctia diastolica

    Pereti ventriculari rigizi

    Functie sistolica pastrata

    Asemanari cu pericardita constrictiva (potential tratabila)

  • 8/14/2019 Curs Studenti Cardiomiopatii

    40/59

    Clasificare

  • 8/14/2019 Curs Studenti Cardiomiopatii

    41/59

    Clasificare

    IdiopaticaMiocardica

    1. Noninfiltrativa

    Idiopatica

    Scleroderma

    2. Infiltrativa

    Amiloid

    Sarcoid

    B. Gaucher

    B. Hurler

    3. B. de stocareHemocromatoza

    B. Fabry

    Stocarea glicogenului

    Endomiocardica

    fibroza endomiocardica

    Sd. Hipereozinofilic

    Sd. carcinoid

    metastaze

    radiatii, antracicline

  • 8/14/2019 Curs Studenti Cardiomiopatii

    42/59

    Tablou clinic

    Simptome de IC dreapta si stanga

    Puls venos jugular

    Curbe x si y proeminente

    Echo-Doppler

    pattern mitral anormal

    E ampla (umplere diastolica rapida)

    TDE scurt (presiune AS crescuta)

    Pattern Constrictiv - Restrictiv

  • 8/14/2019 Curs Studenti Cardiomiopatii

    43/59

    Pattern Constrictiv - RestrictivSquare-Root Sign sau Dip-and-Plateau

    Cateterism cardiac

  • 8/14/2019 Curs Studenti Cardiomiopatii

    44/59

    PTDVS > PTDVD (cu cel putin 5 mmHg)

    PAPs > 50 mmHg

    Platoul PTDVD < 1/3 dinPAPs

  • 8/14/2019 Curs Studenti Cardiomiopatii

    45/59

  • 8/14/2019 Curs Studenti Cardiomiopatii

    46/59

  • 8/14/2019 Curs Studenti Cardiomiopatii

    47/59

    I fi

  • 8/14/2019 Curs Studenti Cardiomiopatii

    48/59

    ImunofixareaAmiloidoz primar cu lanuri uoare de Ig G

    Aspirat din grsimea subcutanat abdominal

  • 8/14/2019 Curs Studenti Cardiomiopatii

    49/59

    p gcoloraie Rou de Congo- depozite de amiloid

    R t i ti C t i ti

  • 8/14/2019 Curs Studenti Cardiomiopatii

    50/59

    Restrictie vs Constrictie

    Pericardita constrictiva

    istoric de TBC, traumatism, pericarditia, colagenoze

    Cardiomiopatia restrictivaamiloidoza, hemocromatoza

    Mixed

    Iradiere mediastinala, chirurgie cardiaca

    Criterii de diagnostic diferenial ecografic ntre pericarditaconstrictiv i cardiomiopatia restrictiv

  • 8/14/2019 Curs Studenti Cardiomiopatii

    51/59

    constrictiv i cardiomiopatia restrictiv

    Pericardita constrictiv Cardiomiopatia restrictiv

    2D - ngroare pericardic, revrsatpericardic

    - dimensiuni normale de perei

    - deplasare abrupt a SIV

    - dilatare biatrial

    - ventriculi de dimensiuninormale

    - hipertrofie de SIV, SIA

    - aspect neomogen al

    miocardului

    Doppler mitral - modificri respiratorii - fr modificri respiratorii

    Velociti inel mitralDoppler tisular (E)

    - >8 cm/s - 50 mmHg( 65 mmHg )

    Tratament

  • 8/14/2019 Curs Studenti Cardiomiopatii

    52/59

    Tratament

    Nu exista terapie eficienta

    diuretice pt presiuni de umplere f mari

    vasodilatoare

    ? Blocantele de calciu pt ameliorarea complianteidiastolice

    digitala si alti agenti inotropi nu sunt indicati

    In cazul amiloidozei agenti alchilanti

  • 8/14/2019 Curs Studenti Cardiomiopatii

    53/59

    Displazia aritmogena de ventriculdrept

    Criteria Major Minor

    Family History Familial disease confirmed at necropsy or Family history of premature suddend h ( 3 ) d b d

  • 8/14/2019 Curs Studenti Cardiomiopatii

    54/59

    surgery death ( 12 in the absence oright bundle branch block

    Tissue characterization of walls Fibrofatty replacement of myocardium onendomyocardial biopsy.

    Global or regional dysfunction andstructural alterations

    Severe dilation and reduction of RVejection fraction with minimal LVinvolvement

    Mild global RV dilation or ejectionfraction reduction with normal LV

    Localized RV aneurysms Mild segmental dilation of the RV

    Severe segmental dilation of the RV Regional RV hypokinesia

    Arrhythmia Left bundle branch lack type ventriculatachycardia (sustained andnonsustained)(ECG, Holter, exersise testing)

    Frequent ventricular extrasystoles (morthan 1,000/24 h) (Holter).

  • 8/14/2019 Curs Studenti Cardiomiopatii

    55/59

  • 8/14/2019 Curs Studenti Cardiomiopatii

    56/59

    Younger patients

    Patients who present with recurrent syncopePatients with history of cardiac arrest or sustained VTPatients with clinical signs of RV failurePatients with LV involvementPatients with or having a family member with the high risk

    ARVD gene (ARVD2)Patients with an increase in QRS dispersion 40 msec

    (maximum measured QRS duration minusminimum measured QRS duration)Patients with Naxos disease

    High Risk Features in Patients with ARVD

  • 8/14/2019 Curs Studenti Cardiomiopatii

    57/59

    Noncompactarea VS

  • 8/14/2019 Curs Studenti Cardiomiopatii

    58/59

  • 8/14/2019 Curs Studenti Cardiomiopatii

    59/59


Recommended