Table 1

Clinical and instrumental characteristics of patients with ALVC according to the clinical scenario

OverallSCDVentricular ArrhythmiasChest PainHeart failureFamiliar Screening
523 (6)15 (29)8 (15)6 (12)20 (38)
Age at diagnosis (years)45 (31–53)23 (19–36)50 (40–57)45 (27–57)47 (46–59)36 (30–50)
Males33 (63)3 (100)11 (73)4 (50%)5 (83)10 (50)
Probands36 (69)3 (100)14 (93)8 (100)5 (83)6 (30)
Family history of sudden death30 (58)010 (67)4 (50)3 (50)13 (65)
 ≤40 years17 (33)03 (20)03 (50)11 (55)
 >40 years13 (25)07 (47)4 (50)02 (10)
Family history of ALVC/ARVC19 (4)02 (13)1 (12)2 (33)14 (70)
Family history of DCM2 (4)01 (6)001 (5)
Any previous myocarditis diagnosis16 (31)1 (17)3 (21)6 (75)4 (67)2 (10)
 Previous myocarditis9 (171 (17)2 (14)4 (50)1 (17)1 (5)
 Instrumental diagnosis of previous myocarditis7 (13)1 (7)2 (25)3 (50)1 (5)
NYHA III/IV2 (4)0001 (17)1 (5)
Echocardiogram51/522 (66)15 (100)8 (100)6 (100)20 (100)
 Phenotype
  Normal21 (41)2 (100)3 (20)4 (50)0 (0)12 (60)
  HNDC13 (25)04 (26)3 (37)2 (33)4 (20)
  DCM17 (33)08 (54)1 (12)4 (67)4 (20)
 LVEF (%)50 (45–57)5749 (43–50)50 (46–62)33 (31–47)53 (49–60)
 LVEF ≤35%2 (4)0002 (33)0
 LVEDV (mL/m2)63 (55–76)6275 (61–80)59 (54–76)81 (70–117)57 (52–60)
 Severe LV dilation4 (8)01 (6)02 (33)1 (5)
High arrhythmic burden30 (57)012 (80)2 (24)4 (67)12 (60)
 VEB >50011 (21)04 (25)1 (12)1 (17)5 (25)
 NSVT19 (36)08 (50)1 (12)3 (50)7 (35)
Cardiac magnetic resonance47158420
 LVEF (%)50 (43–56)48 (41–52)49 (40–57)39 (36–43)53 (51–59)
 LVEDV (mL/m2)101 (88–110)103 (88–119)106 (98–112)102 (98–107)95 (84–109)
 RVEF (%)57 (53–61)56 (53–60)58 (56–60)45 (40–52)59 (54–61)
 RVEDV (mL/m2)83 (68–90)73 (66–82)84 (76–90)89 (72–105)85 (78–91)
 LV fat infiltration23 (49)12 (80)3 (37)1 (25)7
 RV fat infiltration00000
 LGE distribution
  Septum30 (64)9 (60)6 (75)2 (50)13 (65)
  Inferior31 (66)7 (47)7 (87)1 (25)16 (80)
  Infero-lateral30 (64)9 (60)7 (87)2 (50)12 (60)
  Lateral26 (55)6 (43)7 (87)4 (100)9 (45)
  Antero-lateral17 (37)2 (13)6 (75)2 (50)7 (35)
  Anterior21 (45)5 (33)5 (62)2 (50)9 (45)
ECG available50/521 (33)15 (100)8 (100)6 (100)20 (100)
 Normal4 (8)01 (7)1 (17)02 (10)
 T wave inversion
  Isolated in V1-V33 (6)00003 (15)
  Isolated in V4-V6 or/and D1-aVL12 (23)02 (13)2 (25)3 (50)5 (25)
  Isolated in inferior leads2 (4)00002 (10)
  Infero-lateral6 (12)02 (13)1 (12)2 (33)1 (5)
  Diffuse1 (2)0001 (17)0
 Low QRS peripheric voltages23 (47)1 (100)10 (67)3 (37)2 (33)7 (35)
 Q waves13 (52)06 (40)2 (25)2 (33)3 (15)
 LAFB4 (8)001 (12)3 (50)0
 LPFB1 (2)01 (7)000
 LBBB000000
 RBBB3 (6)02 (13)001 (5)
 QRS duration (ms)100 (90–110)95100 (90–114)110 (100–120)110 (100–120)97.5 (88–102)
 QRS≥120 ms6 (12)03 (20)2 (25)2 (33)0
 LVH2 (4)01 (7)1 (12)00
ICD implantation29 (56)9 (60)3 (37)4 (66)13 (65)
 Primary prevention20 (38)2 (13)3 (37)4 (66)11 (55)
 Secondary prevention9 (27)7 (47)002 (10)
  • Values are expressed as n, n (%) or median (IQR).

  • Severe LV dilatation: >100 mL/m2 for male and >80 mL/m2 for female (J Am Soc Echocardiogr 2015;28:1–39).

  • ALVC, arrhythmogenic left ventricle cardiomyopathy; ARVC, arrhythmogenic right ventricle cardiomyopathy; DCM, dilated cardiomyopathy; HNDC, hypokinetic non-dilated cardiomyopathy; ICD, implantable cardioverter defibrillator; LAFB, left anterior fascicular block; LBBB, left bundle branch block; LVEDV, left ventricle end-diastolic volume; LVEF, left ventricle ejection fraction; LVH, left ventricular hypertrophy (one of Sokolow-Lyon, Cornell, Romhilt-Estes, R wave in aVL); NSVT, non-sustained ventricular tachyarrhythmia; NYHA, New York Heart Association; RBBB, right bundle branch block; SCD, sudden cardiac death; TWI, T wave inversion; VEB, ventricular ectopic beats.