SAo subvalvulara:

• CMHO
• SAo discreta

RV CMH, LVNC, CMR, channel 2020 3

Maron B, Maron M, Hypertrophic cardiomyopathy. Lancet 2013
 VD

VS

5
RV
 CMH neobstructiva CMH obstructiva
= =
HVS concentrica sau excentrica HVS + obstructie la ejectia VS
in repaus

la efort
CMH

CMHO
Localizarea si fiziopatologia
obstructiei in CMHO

1.
 Manifestari clinice in CMHO
Simptome
 Asimptomatici
 Dispnee
 Angina (30% din adulti)
 Sincopa (15-25% din cazuri)
 Palpitatii si aritmii V sau SV
 MSC
RV CMH, LVNC, CMR, channel 2020
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
13
EC
G
 Ecocardiografia diastola

 Metoda diagnostica de electie

 HVS:
 Asimetrica: SeIV / PP > 1.5/1 sistola
 Concentrica
 Apicala
 Sept hipertrofiat hipokinetic
 Cavitate VS mica
 SAM a foitei Mi anterioare
 Inchidere mezosistolica a
cuspelor aortice
CM hipertrofica
neobstructiva

Disfunctie diastolica severa

SAM in CMHO - eco transtoracic
CMHO – gradientul intraventricular
CMHO forma apicala
Moon JCC et al. J Am Coll Cardiol 2003
Factorii de risc
Spirito P, et al, NEJM 2000
Maron MS et al. NEJM 2003;348:295-303. RV CMH, LVNC, CMR, channel 2020 28
Sadoul N et al. Circulation 1997
 Stratificare risc

Elliott PM, et al. J Am Coll Cardiol 2000 Elliott PM, et al. Eur Heart J 2006
SCD Risk assessment
ESC 2014
http://doc2do.com/hcm/webHCM.html
Prevention of SCD
Tratamentul CMHO
Alcoolizarea primei septale
Nishimura RA, Holmes DR. NEJM 2004;350:1320.
preoperator postoperator
Heart failure treatment in HCM ESC
2014
Cardiomiopatia prin non-compactare a VS
 Incidence: unknown (0.12 cases per 100,000?); 0.014 to
1.3% of echocardio
 Etiology: genetic (Au dominant > X-linked > Au recessive)
 most commonly runs in a family and affects only the heart.
 cytoskeletal, mitochondrial, sarcomeric and line Z proteins
 Pathogeny:
 altered regulation in cell proliferation, differentiation, and maturation during
ventricular wall formation
 intrauterine arrest of compaction of the loose interwoven meshwork that makes
up the fetal myocardial primordium
 abnormal persistence of the trabecular layer, rather than from noncompaction of
the ventricular wall
 Clinical
 CHF - diastolic or systolic LV dysfunction (typically during the 4th decade
of life, but comorbidities with LV overstrain may anticipate its
expression)
 Thromboembolic complications
 Arrhythmias (ventricular and SV arrhythmias, but also LBBB, AVB and
WPW)
 WPW incidence in iLVNC is higher in children than in adults.
 Prognosis
 high rates of morbidity and mortality, ≈ severity of
disease at clinical presentation
 N heart dimensions and f(x) - negative predictor
SCD
 children>>adults, mortality at 3-4y follow-up 12-
14%!!!)
 Adults:
 annual rate:
 4% c-v deaths
 6.2% c-v death + heart transplantation + appropriate ICD
shocks;
 8.6% all cardiovascular events (death, stroke, implantable
cardioverter-defibrillator shocks, and cardiac transplantation)
 overall mortality rate – 14% at 3y; (1/2 deaths SCD, <1/3
 Treatment
 no specific therapy for LVNC
 Treatment directed by
 clinical manifestations
 LVEF
 Arrhythmias
 risk of thromboembolism