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Relation between QT duration and maximal wall thickness in familial hypertrophic cardiomyopathy
  1. X Jouven1,
  2. A Hagege1,
  3. P Charron2,
  4. L Carrier3,
  5. O Dubourg4,
  6. J M Langlard5,
  7. S Aliaga1,
  8. J B Bouhour5,
  9. K Schwartz3,
  10. M Desnos1,
  11. M Komajda2
  1. 1Service de Cardiologie, Hôpital Européen Georges Pompidou, Paris, France
  2. 2Service de Cardiologie, Hôpital Pitié-Salpêtrière, Paris, France
  3. 3INSERM Unit 153, Paris, France
  4. 4Service de Cardiologie, Hôpital Ambroise Paré, Nantes, France
  5. 5Service de Cardiologie, Hôpital Laennec, Nantes, France
  1. Correspondence to:
    Dr Xavier Jouven, Service de Cardiologie, Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75015 Paris, France;


Background: QT abnormalities have been reported in left ventricular hypertrophy and hypertrophic cardiomyopathy.

Objective: To determine the relation between left ventricular hypertrophy and increased QT interval in familial hypertrophic cardiomyopathy.

Methods: The QT interval was measured in 206 genotyped adult subjects with familial hypertrophic cardiomyopathy from 15 unrelated families carrying mutations in the β myosin heavy chain (β-MHC) gene (five families, n = 68) or the cardiac myosin binding protein C (MyBPC) gene (10 families, n = 138). Subjects were classified as genetically unaffected (controls, n = 112), affected with left ventricular hypertrophy (penetrants, n = 58), or affected without left ventricular hypertrophy (non-penetrants, n = 36).

Results: There was a significant increase in QTmax and QTmin from controls to non-penetrants and penetrants for both the MyBPC group (p ≤ 0.001 and p ≤ 0.001, respectively) and the β-MHC group (p ≤ 0.001 and p ≤ 0.001, respectively). In the MyBPC group, the increase in the QT interval could be explained by increased left ventricular hypertrophy. In the β-MHC group, non-penetrants had a significantly longer QTmax than controls despite the absence of left ventricular hypertrophy, and a similar QT interval to penetrants despite a lesser degree of left ventricular hypertrophy.

Conclusions: In familial hypertrophic cardiomyopathy, genetically affected subjects without left ventricular hypertrophy may have a prolonged QT duration, which depends not only on the degree of left ventricular hypertrophy, when present, but also on the causative mutation.

  • myocardial hypertrophy
  • cardiomyopathy
  • genetics
  • QT duration
  • β-MHC, β-myosin heavy chain
  • MyBPC, cardiac myosin binding protein C
  • QTc, corrected QT
  • QTd, QT dispersion

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