cmh cmr 2015
DESCRIPTION
CMH CMR FloreascaTRANSCRIPT
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 1/54
CARDIOMIOPATIA
HIPERTROFICA
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 2/54
Cardiomiopatia hipertrofica
!
Definitie. CM primara caracterizata prin:"
HVS asimetrica sau concentrica
" Cauza genetica: mutatii ale genelor care
codifica proteinele sarcomerului
"
Disfunctie VS predominent diastolica
!
Cardiomiopatia hipertrofica:
" 75% din cazuri neobstructiva: HVS
" 25% din cazuri obstructiva: HVS +
stenoza aortica subvalvulara
!
Incidenta:
" 0.2 – 0.5% din populatia generala
"
M > F
SAo subvalvulara:• CMHO•
SAo discreta
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 3/54
Incidenta mutatiilor
componentelorsarcomerice in CMH
!
11 gene implicate
! 80% forma familiala
autozomal dominanta
"
Lanturi grele ale
miozinei: cr. 14q1
! 20% forma sporadica
Maron BJ et al. Contemporary definitions and classification of the cardiomyopathies. Circulation 2006;113:1807-16.
cr. 1 cr. 19 cr. 11
cr. 3, 12
cr. 15
cr. 14
cr. 15
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 4/54
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 5/54
CMH:
dezorganizarea cardiomiocitara:
> 5% din masa totala a miocardului
> 20% din zonele hipertrofiate
dezorganizarea miofibrilara
Miocard normal Miocard din CMH
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 6/54
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 7/54
CMH –
anomaliilemicrocirculatiei
pe vase < 1.5 mm
Apare oriunde in miocard
(descrisa initial in zone
cicatriciale sau hipertrofice)
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 8/54
Fiziopatologie: spectrul CMH- HVS inadecvata de cauza genetica -
CMH neobstructiva=
HVS concentrica sau excentrica
CMH obstructiva=
HVS + obstructie la ejectia VS
• 75% din cazurile CMH • 25% din cazurile CMH
•
30% din cazurile CMH •
70% din cazurile CMH
in repaus
la efort
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 9/54
Fiziopatologia CMH
! Tulburarile functiei diastolice:
"
Hipertrofia miocitara
"
Dezordinea miocitara si miofibrilara
"
Fibroza interstitiala
"
Metabolism anormal al calciului"
Ischemia miocardica
! Ischemia miocardica:
" ! rezervei coronare de flux + boala microcirculatiei
#
25% din pacienti au subdenivelari ST la Holter ECG
# 40% au subdenivelari ST la efort
"
Poate fi responsabila de MSC
! +/- obstructie intraventriculara cu gradient dinamic
CMH
CMHO
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 10/54
Localizarea si fiziopatologiaobstructiei in CMHO
!
Proeminenta SIV in LVOT
! Miscare sistolica anterioara
a foitei mitrale anterioare
(SAM)
! Gradient intraventricular
!
Insuficienta mitrala
! Ejectie rapida: VS hiperkinetic
1.
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 11/54
2. 3.
Localizarea si fiziopatologiaobstructiei in CMHO
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 12/54
Manifestari clinice in CMHO
Simptome! Asimptomatici
! Dispnee
! Angina (30% din adulti)
! Sincopa (15-25% din cazuri)
!
Palpitatii si aritmii V sau SV
! MSC
Semne!
“Pulsus bisferiens”
!
Soc apexian hiperdinamic
!
Galop presistolic!
SUFLU DE EJECTIE variabil:
"
" prin ! volumului VS (efort,
tahicardie, nitrit de amil, Valsalva,
ortostatism)
"
! prin " volumului VS (PS
mimetice, pozitia “pe vine”)
! Suflu de insuficienta mitrala
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 13/54
ECG
! HVS cu gradient alterat
!
T negative in precordiale
!
Unde Q septale: 25-50% din
cazuri
! P mitral
! Aritmii atriale sau ventric.
! T negative gigante in V1-V6
in CMH apicala
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 14/54
Ecocardiografia
diastola
sistola
!
Metoda diagnostica de electie
! HVS:
"
Asimetrica: SeIV / PP > 1.5/1
"
Concentrica"
Apicala
! Sept hipertrofiat hipokinetic
! Cavitate VS mica
! SAM a foitei Mi anterioare
! Inchidere mezosistolica a
cuspelor aortice
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 15/54
CM hipertrofica
neobstructiva
Disfunctie diastolica severa
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 16/54
SAM in CMHO - eco transtoracic
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 17/54
SAM in CMHO: TEE
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 18/54
CMHO –
gradientul
intraventricular
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 19/54
CMHO forma apicala
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 20/54
Cateterismul cardiac: gradientul intraventricular
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 21/54
CMHO angio
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 22/54
CMHO – SAM angio
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 23/54
Moon JCC et al. J Am Coll Cardiol 2003
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 24/54
Factorii de risc
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 25/54
TVNS
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 26/54
Factori de risc: vârsta
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 27/54
Factori de risc: grosimea peretelui
Spirito P, et al, NEJM 2000
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 28/54
Valoarea prognostica a gradientuluiintraventricular in repaus > 30 mmHg
Maron MS et al. NEJM 2003;348:295-303.
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 29/54
raspunsul TA la efort
Sadoul N et al. Circulation 1997
Stratificare risc
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 30/54
Stratificare risc
Elliott PM, et al. Eur Heart J 2006Elliott PM, et al. J Am Coll Cardiol 2000
SCD Ri k t
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 31/54
SCD Risk assessmentESC 2014
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 32/54
Prevention of SCD
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 33/54
Prevention of SCD
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 34/54
Tratamentul CMHO
!
Evitarea efortului fizic: 40% din MSC se produc la efort!
Ameliorarea simptomelor = reducerea gradientului
intraventricular
"
Inotrop negative: #-blocante, disopiramida, verapamil
"
Embolizarea septala
"
Pacing bicameral / tricameral
"
Miectomie chirurgicala
!
Preventia mortii subite la pacientii cu risc inalt:
"
Reducerea gradientului IV
"
Amiodarona, defibrilator implantabil
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 35/54
Alcoolizarea primei septale
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 36/54
Miotomia-miectomia Morrow in CMHO
Nishimura RA, Holmes DR. NEJM 2004;350:1320.
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 37/54
Miotomia-miectomia Morrow in
CMHO
preoperator postoperator
f l C
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 38/54
Heart failure treatment in HCMESC 2014
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 39/54
CARDIOMIOPATIILE
RESTRICTIVE
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 40/54
CMR
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 41/54
CMR:anatomie patologica
HE x 250
Hipertrofie miocitara usoara
HE x 40
Fibroza interstitiala marcata
Depinde de etiologie
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 42/54
Fiziopatologie
!
" rigiditatii miocardului sau
endocardului
!
" rapida a presiunii de umplere
ventriculare
! Aspect caracteristic al curbei de
presiune diastolice:
“dip and plateau”
!
Afectare predominanta a VD (+/- VS)
! Dilatatie atriala marcata cu cavitati
ventriculare normale
! Semne predominente de IVD: " PVC
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 43/54
Diferente intre CMR si pericarditaconstrictiva
CMR Pericarditaconstrictiva
Presiuni diastolice A-V Diferenta > 5 mmHg Diferenta < 5 mmHg
HTP """ (PAPs > 50 mmHg) " sau $
Platou diastolic alpresiunii VD
< 1/3 din presiuneasistolica
> 1/3 din presiuneasistolica
Biopsiaendomiocardica
Anormala Normala
BNP > 5 x N N
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 44/54
Manifestari clinice
!
Se coreleaza cu severitatea " presiunii in atrii" Intoleranta la efort
#
Incapacitatea cresterii DC prin disfunctia diastolica
" Dispnee
"
Astenie severa = ! DC
! Semne de PVC mare:
" turgescenta jugulara, hepatomegalie, ascita, edeme, anasarca
" Instalare progresiva, greu reversibile
!
Fibrilatie atriala = frecventa prin dilatatie atriala
! Aritmii ventriculare frecvente in faze avansate = cauza deces
! Complicatii tromboembolice la 1/3 din cazuri
! DIAGNOSTIC: ecocardiografie, cateterism cardiac, biopsie endomiocardica
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 45/54
CMR – aspect ecocardiografic
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 46/54
Tratamentul CMR
!
Identificarea cauzelor tratabile (ex. hemocromatoza,carcinoid)
! Diuretice ptr reducerea PVC si a retentiei hidrosaline
!
IECA determina hipoTA
! Digitalice: inutile patogenic, toxicitate crescuta
! Aritmii ventriculare grave:
"
defibrilator implantabil,
"
beta blocante sau
"
transplant
! Transplant cardiac (nu in bolile sistemice)
E d dit
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 47/54
EndocarditaLoffler
Infiltrare miocardica extensivacu eozinofile
" Caracteristica zonelor temperate
"
Eozinofilie sanguina si miocardica
marcata
" M < 50 ani cu hipereozinofilie
" Etape clinice succesive:
" Necrotica
" Trombotica
" Fibrotica
"
Semne de IC congestiva + embolii
sistemice
" Tratament: corticosteroizi,
hidroxiuree, transplant cardiac
b d d
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 48/54
Fibroza endomiocardicao Caracteristica zonelor tropicaleo Eozinofilie usoara
o Fibroza endocardica apicala si subvalvulara
o Afectare biventriculara 50%; VS = 40%o 15-25% din decese cardiace ale zoneio Afecteaza copii si adulti tinerio Mortalitate la 2 ani: 35-50%
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 49/54
Amiloidoza miocardica! CMR cea mai frecvent intalnita in practica
!
Boala infiltrativa multisistemica cu depunere de fibrile polipeptidice liniare ininterstitiul miocardic:
" Lanturi usoare de Ig (kappa sau lambda): forma AL = forma primara, varianta de mielom
" Non Ig (proteina A): forma AA = forma secundara
" Forma familiala (AF): prealbumina (transtiretina)
" Forma senila (SSA): ANP-like sau transtiretina
! Aparitia manifestarilor clinice = infiltrare severa
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 50/54
Amiloidoza
cutaneomucoasa
manifestari neurologice
Depunerea in rinichi
= sdr. nefrotic
Amiloid: termenul lui Virchow“amidon-like”
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 51/54
Amiloidoza miocardica
ECG: microvoltaj, tulburari de conducere AV sau BR
Afectarea cardiaca = 1/3 pts cu amiloidoza sistemica tip AL
Afectarea anatomopatologica este prezenta si in lipsa manifestarilor clinice
intereseaza miocardul V, A si eventual si valvele
manifestari clinice: CMR, IC sistolica, hipoTA ortostatica, BAV
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 52/54
Amiloidoza miocardica
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 53/54
Aspectul
ecocardiografic inamiloidoza
7/21/2019 Cmh Cmr 2015
http://slidepdf.com/reader/full/cmh-cmr-2015 54/54
Tratamentul amiloidozei
! Amiloidoza AL = prognostic c.m. infaust
! Agenti alkilanti: melfalan
! Imunosupresie: prednison
! Toxice ale fusului: colchicina
! Tratamentul IC:
"
Evitarea digitalicelor (afinitate ptr fibrele de amiloid = toxicitate)
"
Sensibilitate majora la Ca blocante
"
Vdil si diureticele: efecte hipotensoare"
ANTICOAGULARE sistemica
! Transplant cardiac in formele fara afectare extracardiaca
! Transplant hepatic in formele cu transtiretina