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Cardiomyopathie hypertrophique obstructive Echoca
rdiographie
Luc A. Piérard , CHU Liège
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Bénigne et Stable
Pronostic CMH
Progression des symptômes
Ins. cardiaque
Mort Subite
FA
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STRATIFICATION DU RISQUE GENETIQUE Histoire
familiale de mort subite Mutations spécifiques
CLINIQUE Arrêt cardiaque réanimé TV soutenue
(gt30 s) spontanée Syncopes récidivantes TV au
Holter MORPHOLOGIQUE HVG sévère ( gt 3
CM) HEMODYNAMIQUE Gradient chambre de
chasse( gt 30 mm Hg) Chute de PA à leffort
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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PHYSIOPATHOLOGIE - Trouble de la relaxation
VG - Perte de compliance ventriculaire -
Fonction systolique normale ou accrue ( FE gt 80
) - Ejection surtout protosystolique (80
pendant la 1ère moitié) - Gradient possible
labile au niveau sous-valvulaire - chambre de
chasse VG (SAM) - région médioventriculaire
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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 2003348295-303
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HCM- RELATED DEATH VARIABLE RELATIVE
RISK p VALUE (95 CI) LV OUTLOW
OBSTRUCTION ( gt 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 gt 30 mm
1.8 (1.1 - 2.8) 0.01 FEMALE SEX -
0.29
Maron et al NEJM 2003348295-303
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Maron et al NEJM 2003348295-303
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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 ( gt 30 mm Hg) 2.7 (2.0 - 3.5)
lt 0.001 NYHA CLASS II, III, OR IV AT ENTRY
3.4 (2.4 - 4.8) lt
0.001 PAROXYSMAL OR CHRONIC ATRIAL AF 1.3
(1.1 - 1.6) 0.046 MAXIMAL LV THICKNESS gt 30 mm
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0.09 FEMALE SEX 1.4 (1.1 - 1.8) 0.02
Maron et al NEJM 2003348295-303
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Maron et al NEJM 2003348295-303
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SUDDEN DEATH FROM
HCM VARIABLE RELATIVE RISK p
VALUE (95 CI) LV
OUTLOW OBSTRUCTION ( gt 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 gt 30 mm
- 0.82 FEMALE SEX
- 0.75
Maron et al NEJM 2003348295-303
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Maron et al NEJM 2003348295-303
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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 gt 30 - 50 mmHg
Provocable mild gradient at rest gradient gt
30 - 50 mmHg with provocation Latent no
gradient at rest significant gradient with
provocation Nonobstructive gradient lt 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. gt3m/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 gt 50 mmHg at rest 201 pts
exercise echo 106 (52) dynamic obstruction
gt 30 mmHg 76 (38) substantial gradient gt 50
mmHg 95 (47) nonobstructive form (lt 30
mmHg) Thus 225/320 pts (70) outflow
obstruction Implications more candidates for
septal reduction therapy ??
Maron et al Circulation 20061142232-9
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Maron et al Circulation 20061142232-9
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Maron et al Circulation 20061142232-9
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Maron et al Circulation 20061142232-9
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CONCLUSIONS
Obstruction to LV outflow has prognostic
importance No role of stress testing when
baseline gradient gt 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
DDD Pacing
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 linfarctus induit .
élimination de lobstacle à léjection .
hospitalisation courte . mortalité 2 (taux
similaire à celui de la chirurgie) . bloc AV
complet 25,nécessitant stimulateur . rarement
infarctus massif . courbe dapprentissage -
Effets morphologiques différents
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SURGICAL MYECTOMY vs ALCOHOL SEPTAL ABLATION
Cine and contrast-enhanced CMR before and
after septal myectomy (n24) septal ablation
(n24) Myectomyresected 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 200749350-7