curs studenti cardiomiopatii
TRANSCRIPT
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Cardiomiopatiile
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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.
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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)
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Cardiomiopatia Dilatativa Idiopatica
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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 !
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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
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Supravietuirea observata in cadrul unui lotde 104 pacienti
YearsAm J Cardiol 1981; 47:525
Cardiomiopatia dilatativa idiopatica
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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 !
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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
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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
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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
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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
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Examinari de laborator
ECG
Rx cardio-pulmonara Holter ECG (ameteala, palpitatii, sincope) Ecocardiografie Cateterism cardiac
F t i l ti i CMD
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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
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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
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Managementul CMD
Tratament imunosupresivResincronizareTratament chirurgical (valva mitrala, remodelare VS)
Dispozitive de asistare ventricularaTransplant cardiac
Indicatii clinice ale biopsiei
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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)
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Cardiomiopatia hipertrofica (CMH)
C di i ti hi t fi
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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)
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Sept si perete anterolateral VS - frecvent Perete posterobazal - rar
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CMH - Histologie
Hipertrofie miocitara
Dezorganizare miocitara disarray > 5% din miocard
CMH Fiziopatologie
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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
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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
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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
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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
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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
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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
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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
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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
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CMH - Ecocardiografie
SIV si/sau PP > 15 mm 60 mm
VMA lungaproemina inTEVS
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Istoria naturala
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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
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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
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Recomandari pentru activitate competitiona
Evitarea sporturilor competiotionale indiferentdaca exista sau nu gradient dinamic
CMH - Management
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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
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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
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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
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Cardiomiopatia restrictiva
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Cardiomiopatia restrictiva
Trasatura definitorie: disfunctia diastolica
Pereti ventriculari rigizi
Functie sistolica pastrata
Asemanari cu pericardita constrictiva (potential tratabila)
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Clasificare
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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
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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
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Pattern Constrictiv - RestrictivSquare-Root Sign sau Dip-and-Plateau
Cateterism cardiac
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PTDVS > PTDVD (cu cel putin 5 mmHg)
PAPs > 50 mmHg
Platoul PTDVD < 1/3 dinPAPs
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I fi
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ImunofixareaAmiloidoz primar cu lanuri uoare de Ig G
Aspirat din grsimea subcutanat abdominal
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p gcoloraie Rou de Congo- depozite de amiloid
R t i ti C t i ti
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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
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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
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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
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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
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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).
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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
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Noncompactarea VS
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