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Luc A. Piérard , CHU Liège

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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  1. Cardiomyopathie hypertrophique obstructive Echocardiographie Luc A. Piérard , CHU Liège

  2. Bénigne et Stable Pronostic CMH Progression des symptômes Ins. cardiaque Mort Subite FA

  3. 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

  4. ECHOCARDIOGRAMME - Hypertrophie septale asymétrique - Distribution variable - Parfois hypertrophie exclusivement apicale - Mouvement systolique antérieur de la valve mitrale (SAM) - Fermeture précoce de la valve aortique

  5. 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

  6. Maron et al NEJM 2003;348:295-303

  7. 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 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

  8. Maron et al NEJM 2003;348:295-303

  9. 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

  10. Maron et al NEJM 2003;348:295-303

  11. 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

  12. Maron et al NEJM 2003;348:295-303

  13. CONSEQUENCES OF CHRONIC OUTFLOW GRADIENT Increase in LV wall stress Myocardial ischaemia Cell death Fibrosis

  14. 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

  15. INTERVENTIONS TO INDUCE GRADIENTS Amyl nitrite inhalation Valsalva maneuver Post-PVC response Isoproterenol infusion Dobutamine infusion Standing posture Physiologic exercise (during and after)

  16. GRADIENT MAJORE APRES EXTRASYSTOLE

  17. GRADIENT MAJORE PENDANT MANŒUVRE DE VALSALVA

  18. DOBUTAMINE STRESS ECHO

  19. DOBUTAMINE INFUSION

  20. 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

  21. 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

  22. EXERCISE FOR DEFINING LATENT OBSTRUCTION Immediately following treadmill or bicycle exercise During and immediately after semi-supine exercise No drug withdrawal

  23. Exercise Echo in HCM

  24. 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

  25. Maron et al Circulation 2006;114:2232-9

  26. Maron et al Circulation 2006;114:2232-9

  27. Maron et al Circulation 2006;114:2232-9

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