Familial Hypertrophic Cardiomyopathy With Triphasic Transmitral Flow Velocity

抄録

We describe a family in which five members had hypertrophic cardiomyopathy (HCM) with a similar distribution of left ventricular (LV) hypertrophy and LV filling pattern. All five family members had myocardial hypertrophy localized between the anterior LV wall and ventricular septum, in addition to having a triphasic transmitral flow (TMF) velocity pattern with a mid-diastolic wave. The HCM patients with this TMF velocity pattern exhibit decreased LV compliance and delayed LV relaxation, suggesting that the features of diastolic failure may be specific. Thus, these findings may provide an important clue to elucidating the relationship between the pathophysiology and mode of inheritance in patients with HCM.