Table 1

Echocardiographic Features of Affected Family Members From Families 1 and 2

Subject No.Two-Dimensional Echocardiogram
LVWT
Age (yrs)/GenderVS (mm)PW (mm)LVEDD (mm)LVESD (mm)EF (%)
Family 1
 IV-0116/M8125534Normal
 IV-021/M*1113403610
 IV-035/M*111257545
 IV-046/M983824Normal
Family 2
 III-1545/F18134636Normal
 IV-0134/M1811696010
 IV-0339/F16134733Normal
 IV-0536/M18234733Normal
 IV-0831/F17174024Normal
 IV-0922/M27255234Normal
232516827217
 IV-2027/M19164933Normal
281813685640
 IV-2330/M13165233Normal
 V-0518/F13154532Normal
 V-0717/F13164633Normal
 V-0814/M12154632Normal
 V-1310/M1274531Normal
 V-1410/F12154532Normal
 V-2010/F1294632Normal

LVEDD = left ventricular end-diastolic diameter; LVESD = left ventricular end-systolic diameter; LVWT = left ventricular wall thickness; PW = posterior wall; VS = ventricular septum; other abbreviations as in Table 1.

  • * Age at time of death. Family 1: The measurements shown for subject nos. IV-01 and IV-04 represent the most recent data, whereas those for IV-02 and IV-03 were taken on admission during the final illness. Family 2: Two values are given for subject nos. IV-09 and IV-20, indicating the rapid decline in cardiac function observed in these patients.