luc a. piérard , chu liège
DESCRIPTION
Cardiomyopathie hypertrophique obstructive Echocardiographie. Luc A. Piérard , CHU Liège. Bénigne et Stable. Pronostic CMH. Progression des symptômes. Ins. cardiaque. Mort Subite. FA. STRATIFICATION DU RISQUE GENETI QUE Histoire familiale de mort subite Mutations spécifiques - PowerPoint PPT PresentationTRANSCRIPT
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Luc A. Piérard , CHU Liège
Cardiomyopathie hypertrophique obstructive
Echocardiographie
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Pronostic CMH
Bénigne et Stable
MortSubite
FA Progressiondes symptômes
Ins. cardiaque
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STRATIFICATION DU RISQUE
GENETIQUEHistoire familiale de mort subiteMutations spécifiques
CLINIQUEArrêt cardiaque réaniméTV soutenue (>30 s) spontanéeSyncopes récidivantesTV au Holter
MORPHOLOGIQUE HVG sévère ( > 3 CM)
HEMODYNAMIQUE Gradient chambre de chasse( > 30 mm Hg)
Chute de PA à l’effort Réserve coronaire réduite
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ECHOCARDIOGRAMME
- Hypertrophie septale asymétrique
- Distribution variable
- Parfois hypertrophie exclusivement apicale
- Mouvement systolique antérieur de la valve mitrale (SAM)
- Fermeture précoce de la valve aortique
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ECHO DOPPLER
Formes obstructives
- Accélération du flux dans la chambre de chasse
- Maximum télésystolique : aspect en « lame de sabre »
- Gradient = 4 V2
- Régurgitation mitrale associée
- Variations du gradient en cas de pré- et post-charge (nitré)
Fonction diastolique
Etude du remplissage VG et Doppler tissulaire
. Trouble de relaxation
vs
. compliance
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Maron et al NEJM 2003;348:295-303
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HCM- RELATED DEATHVARIABLE
RELATIVE RISK p VALUE(95 % CI)
LV OUTLOW OBSTRUCTION ( > 30 mm Hg) 1.6 (1.1 - 2.4) 0.02
NYHA CLASS II, III, OR IV AT ENTRY 1.9 (1.2 - 2.9) 0.002
PAROXYSMAL OR CHRONIC ATRIAL AF 1.6 (1.1 - 2.4) 0.01
MAXIMAL LV THICKNESS > 30 mm 1.8 (1.1 - 2.8) 0.01
FEMALE SEX - 0.29
Maron et al NEJM 2003;348:295-303
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Maron et al NEJM 2003;348:295-303
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HCM- RELATED PROGRESSION TO NYHA
VARIABLE CLASS III OR IV OR DEATH FROM HEART FAILURE OR STROKE
RELATIVE RISK p VALUE (95 % CI)
LV OUTLOW OBSTRUCTION ( > 30 mm Hg) 2.7 (2.0 - 3.5) < 0.001
NYHA CLASS II, III, OR IV AT ENTRY 3.4 (2.4 - 4.8) < 0.001
PAROXYSMAL OR CHRONIC ATRIAL AF 1.3 (1.1 - 1.6) 0.046
MAXIMAL LV THICKNESS > 30 mm - 0.09
FEMALE SEX 1.4 (1.1 - 1.8) 0.02
Maron et al NEJM 2003;348:295-303
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Maron et al NEJM 2003;348:295-303
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SUDDEN DEATH FROM HCM
VARIABLE RELATIVE RISK p VALUE
(95 % CI)
LV OUTLOW OBSTRUCTION ( > 30 mm Hg) 1.9 (1.1 - 3.5) 0.014
NYHA CLASS II, III, OR IV AT ENTRY - 0.12
PAROXYSMAL OR CHRONIC ATRIAL AF - 0.72
MAXIMAL LV THICKNESS > 30 mm - 0.82
FEMALE SEX - 0.75
Maron et al NEJM 2003;348:295-303
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Maron et al NEJM 2003;348:295-303
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CONSEQUENCES OF CHRONIC OUTFLOW GRADIENT
Increase in LV wall stress
Myocardial ischaemia
Cell death
Fibrosis
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HAEMODYNAMIC SUBGROUPS IN HCM
Obstructive : gradient at rest > 30 - 50 mmHg
Provocable :mild gradient at restgradient > 30 - 50 mmHg with provocation
Latent :no gradient at restsignificant gradient with provocation
Nonobstructive :gradient < 30 mmHg under basal and provocable conditions
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INTERVENTIONS TO INDUCE GRADIENTS
Amyl nitrite inhalation
Valsalva maneuver
Post-PVC response
Isoproterenol infusion
Dobutamine infusion
Standing posture
Physiologic exercise (during and after)
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GRADIENT MAJORE APRES EXTRASYSTOLE
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GRADIENT MAJORE PENDANT MANŒUVRE DE VALSALVA
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DOBUTAMINE STRESS ECHO
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DOBUTAMINE INFUSION
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LV OBSTRUCTION DURING DOBUTAMINE STRESS ECHO
232 consecutive pts : normal DSE (no HCM)
31 pts (13%):LVOT vel. >3m/s (36 mmHg)
7 unable toexercise
24 underwentEx stress echo
Possible angina : 19
Dyspnea : 4
Syncope : 1
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DSE vs Ex SE IN 24 PATIENTS
17 women , 7 men
Hypertension in 12 pts
LVOT diameter : 22 ± 2 mm (18-25 mm)
Basal septal diastolic thickness : 13 ± 2 mm (9-15 mm)
Peak velocity with Dobutamine : 4 ± 0.8 m/s (3-6.3)
Peak velocity with Exercise : range 0.9 to 2.2 m/s
No patient developed LV gradient
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EXERCISE FOR DEFINING LATENT OBSTRUCTION
Immediately following treadmill or bicycle exercise
During and immediately after semi-supine exercise
No drug withdrawal
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Exercise Echo in HCM
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EXERCISE ECHO IN HCM
320 consecutive patients
119 pts (37%) : LV outflow tract gradient > 50 mmHg at rest
201 pts : exercise echo106 (52%) : dynamic obstruction > 30 mmHg 76 (38%) : substantial gradient > 50 mmHg 95 (47%) : nonobstructive form (< 30 mmHg)
Thus : 225/320 pts (70%) : outflow obstruction
Implications : more candidates for septal reduction therapy ??
Maron et al Circulation 2006;114:2232-9
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Maron et al Circulation 2006;114:2232-9
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Maron et al Circulation 2006;114:2232-9
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Maron et al Circulation 2006;114:2232-9
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CONCLUSIONS
Obstruction to LV outflow has prognostic importance
No role of stress testing when baseline gradient > 30-50 mmHg
Preferred provocative maneuver : exercise
Measurement of gradient mandatory during and after exercise
Dobutamine stress testing should not be used
The prognostic importance of provocable obstructionremains unknown
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SYMPTOMS
DrugsBeta-blockerVerapamilDisopyramide
Drugrefractorysymptoms
Obstructive HCM(rest or provocation)
Alternativesto surgery
SurgerySeptal myectomy
DDDPacing
Alcoholseptal ablation
Non-ostructive HCM(rest and provocation
End-stage HF treatment ,heart transplant
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TRAITEMENT DE L ’OBSTRUCTION SYMPTOMATIQUE
- Chirurgie : myotomie + myectomie septale haute
- Alcoolisation de la première septale :
. épaisseur à cause de l’infarctus induit
. élimination de l’obstacle à l’éjection
. hospitalisation courte
. mortalité 2% (taux similaire à celui de la chirurgie)
. bloc AV complet 25%,nécessitant stimulateur
. rarement infarctus massif
. courbe d’apprentissage
- Effets morphologiques différents
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SURGICAL MYECTOMY vs ALCOHOL SEPTAL ABLATION
Cine and contrast-enhanced CMR before and afterseptal myectomy (n=24)septal ablation (n=24)
Myectomy:resected tissue always localized to anterior septum
Ablation: more variable effect transmural tissue necrosis,more inferiorly in basal septum extending into RV side of septum at mid-ventricular level 6 pts: sparing of the basal septum with residual gradient
LBBB in 46% after myectomyRBBB in 58% after ablation
8 of 47 pts(17%) :heart block requiring PMK (excluded)Valeti et al JACC 2007;49:350-7