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Tableau I.
Résumé des données cliniques.
| CMH (n = 17) | CMD (n = 9) | CMR (n = 4) | |
|---|---|---|---|
| Âge moyen au diagnostic (ans) | 33 [9-66] | 32 [0-51] | 36 [23-46] |
| FEVG moyen (%) | Normal | 37 ± 6 % | Normal + Profil restrictif |
| Épaisseur du septum interventriculaire (mm) | 15,8 ± 3,0 | NA | NA |
| Épaisseur de la paroi postérieure (mm) | 18,5 ± 4,4 | NA | NA |
| Appareillage (DAI/PM) | 3/17 | 2/9 | 1/4 |
| Histoire de mort subite personnelle ou familiale | 2/17 | 3/9 | ¾ patients décédés ou en attente de greffe |
| Anomalies ECG *FA *Troubles conduction | 4/17 6/17 | 1/9 2/9 | 1/4 |
| CK | normales | normales | normales |
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