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Table 1 Clinical characteristics in family ALB

From: Exome sequencing identifies a mutation in the ACTN2 gene in a family with idiopathic ventricular fibrillation, left ventricular noncompaction, and sudden death

ID Gender Current age (yrs) Age at echo (yrs) LVmax (mm) PW (mm) LVEDD (mm) EF (%) ECG ICD Appropriate ICD shocks Final diagnosis
II-3 M Deceased 60 12 12 58 27 LBBB No NA DCM
III-2 F 35 29 6 6 51 N Normal No NA Normal
III-3 F 33 27 6 6 49 N Minor T-wave changes Yes 2 IVF (RCA)
III-5 F 35 34 6 4 51 N ST-T wave changes Yes 0 LVNC
III-6 F Died 25 NA NA NA NA NA NA No NA SUDY
  1. LVmax - maximum left ventricular wall thickness; PW - posterior wall thickness; LVEDD – left ventricular end-diastolic diameter; EF – ejection fraction; ICD – implantable cardioverter-defibrillator; ECG – electrocardiogram; LBBB – left bundle branch block; DCM – dilated cardiomyopathy; RCA – resuscitated cardiac arrest; IVF – idiopathic ventricular fibrillation; LVNC – left ventricular non-compaction; SUDY – sudden unexplained death in the young; N – normal; NA – not applicable.