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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

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Luc A. Piérard , CHU Liège

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Luc a pi rard chu li ge

Cardiomyopathie hypertrophique obstructive

Echocardiographie

Luc A. Piérard , CHU Liège


Luc a pi rard chu li ge

Bénigne et Stable

Pronostic CMH

Progression

des symptômes

Ins. cardiaque

Mort

Subite

FA


Luc a pi rard chu li ge

STRATIFICATION DU RISQUE

GENETIQUE

Histoire familiale de mort subite

Mutations spécifiques

CLINIQUE

Arrêt cardiaque réanimé

TV soutenue (>30 s) spontanée

Syncopes récidivantes

TV 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 DEATH

VARIABLE

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 AF1.6 (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


Luc a pi rard chu li ge

Maron et al NEJM 2003;348:295-303


Luc a pi rard chu li ge

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


Luc a pi rard chu li ge

Maron et al NEJM 2003;348:295-303


Luc a pi rard chu li ge

SUDDEN DEATH

FROM HCM

VARIABLE

RELATIVE RISKp 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


Luc a pi rard chu li ge

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 rest

gradient > 30 - 50 mmHg with provocation

Latent :

no gradient at rest

significant 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)

Possible angina : 19

Dyspnea : 4

Syncope : 1

7 unable to

exercise

24 underwent

Ex stress echo


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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 echo

106 (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 obstruction

remains unknown


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SYMPTOMS

Beta-blocker

Verapamil

Disopyramide

Drugs

Drug

refractory

symptoms

Obstructive HCM

(rest or provocation)

Alternatives

to surgery

Surgery

Septal myectomy

Alcohol

septal ablation

Non-ostructive HCM

(rest and provocation

DDD

Pacing

End-stage

HF treatment ,heart transplant


Luc a pi rard chu li ge

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 after

septal 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 myectomy

RBBB in 58% after ablation

8 of 47 pts(17%) :heart block requiring PMK (excluded)

Valeti et al JACC 2007;49:350-7


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