Table 1

Clinical, ECG, and echocardiographic characteristics of affected subjects

NoAge (y)Sex (M/F)ECGMax IVS (mm)Spirito score (mm)Maron typeGene mutation
I.384FVentricular pacing2567IIIMYH7
II.156FLVH, negative T waves3285IIIMYH7/MYBPC3
II.258MNormal1549IIMYH7
II.362FNormal2161IIIMYH7
III.130FNegative T waves (V2-4)2063IIIMYBPC3
III.331MNegative T waves (V2-4)1963IIIMYH7
III.533MLVH, negative T waves2875IIIMYH7/MYBPC3
III.827FNormal1752IIIMYBPC3
  • Max IVS: maximal end diastolic interventricular wall thickness. Maron type: type II hypertrophy involving both the anterior and posterior segments of the interventricular septum, type III hypertrophy involving both the interventricular septum and the left ventricular free wall.