Table 1.
Clinical and instrumental characteristics of patients with ALVC according to the clinical scenario
| Overall | SCD | Ventricular Arrhythmias | Chest Pain | Heart failure | Familiar Screening | |
| 52 | 3 (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) |
| Males | 33 (63) | 3 (100) | 11 (73) | 4 (50%) | 5 (83) | 10 (50) |
| Probands | 36 (69) | 3 (100) | 14 (93) | 8 (100) | 5 (83) | 6 (30) |
| Family history of sudden death | 30 (58) | 0 | 10 (67) | 4 (50) | 3 (50) | 13 (65) |
| ≤40 years | 17 (33) | 0 | 3 (20) | 0 | 3 (50) | 11 (55) |
| >40 years | 13 (25) | 0 | 7 (47) | 4 (50) | 0 | 2 (10) |
| Family history of ALVC/ARVC | 19 (4) | 0 | 2 (13) | 1 (12) | 2 (33) | 14 (70) |
| Family history of DCM | 2 (4) | 0 | 1 (6) | 0 | 0 | 1 (5) |
| Any previous myocarditis diagnosis | 16 (31) | 1 (17) | 3 (21) | 6 (75) | 4 (67) | 2 (10) |
| Previous myocarditis | 9 (17 | 1 (17) | 2 (14) | 4 (50) | 1 (17) | 1 (5) |
| Instrumental diagnosis of previous myocarditis | 7 (13) | – | 1 (7) | 2 (25) | 3 (50) | 1 (5) |
| NYHA III/IV | 2 (4) | 0 | 0 | 0 | 1 (17) | 1 (5) |
| Echocardiogram | 51/52 | 2 (66) | 15 (100) | 8 (100) | 6 (100) | 20 (100) |
| Phenotype | ||||||
| Normal | 21 (41) | 2 (100) | 3 (20) | 4 (50) | 0 (0) | 12 (60) |
| HNDC | 13 (25) | 0 | 4 (26) | 3 (37) | 2 (33) | 4 (20) |
| DCM | 17 (33) | 0 | 8 (54) | 1 (12) | 4 (67) | 4 (20) |
| LVEF (%) | 50 (45–57) | 57 | 49 (43–50) | 50 (46–62) | 33 (31–47) | 53 (49–60) |
| LVEF ≤35% | 2 (4) | 0 | 0 | 0 | 2 (33) | 0 |
| LVEDV (mL/m2) | 63 (55–76) | 62 | 75 (61–80) | 59 (54–76) | 81 (70–117) | 57 (52–60) |
| Severe LV dilation | 4 (8) | 0 | 1 (6) | 0 | 2 (33) | 1 (5) |
| High arrhythmic burden | 30 (57) | 0 | 12 (80) | 2 (24) | 4 (67) | 12 (60) |
| VEB >500 | 11 (21) | 0 | 4 (25) | 1 (12) | 1 (17) | 5 (25) |
| NSVT | 19 (36) | 0 | 8 (50) | 1 (12) | 3 (50) | 7 (35) |
| Cardiac magnetic resonance | 47 | – | 15 | 8 | 4 | 20 |
| 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 infiltration | 23 (49) | 12 (80) | 3 (37) | 1 (25) | 7 | |
| RV fat infiltration | 0 | 0 | 0 | 0 | 0 | |
| LGE distribution | ||||||
| Septum | 30 (64) | 9 (60) | 6 (75) | 2 (50) | 13 (65) | |
| Inferior | 31 (66) | 7 (47) | 7 (87) | 1 (25) | 16 (80) | |
| Infero-lateral | 30 (64) | 9 (60) | 7 (87) | 2 (50) | 12 (60) | |
| Lateral | 26 (55) | 6 (43) | 7 (87) | 4 (100) | 9 (45) | |
| Antero-lateral | 17 (37) | 2 (13) | 6 (75) | 2 (50) | 7 (35) | |
| Anterior | 21 (45) | 5 (33) | 5 (62) | 2 (50) | 9 (45) | |
| ECG available | 50/52 | 1 (33) | 15 (100) | 8 (100) | 6 (100) | 20 (100) |
| Normal | 4 (8) | 0 | 1 (7) | 1 (17) | 0 | 2 (10) |
| T wave inversion | ||||||
| Isolated in V1-V3 | 3 (6) | 0 | 0 | 0 | 0 | 3 (15) |
| Isolated in V4-V6 or/and D1-aVL | 12 (23) | 0 | 2 (13) | 2 (25) | 3 (50) | 5 (25) |
| Isolated in inferior leads | 2 (4) | 0 | 0 | 0 | 0 | 2 (10) |
| Infero-lateral | 6 (12) | 0 | 2 (13) | 1 (12) | 2 (33) | 1 (5) |
| Diffuse | 1 (2) | 0 | 0 | 0 | 1 (17) | 0 |
| Low QRS peripheric voltages | 23 (47) | 1 (100) | 10 (67) | 3 (37) | 2 (33) | 7 (35) |
| Q waves | 13 (52) | 0 | 6 (40) | 2 (25) | 2 (33) | 3 (15) |
| LAFB | 4 (8) | 0 | 0 | 1 (12) | 3 (50) | 0 |
| LPFB | 1 (2) | 0 | 1 (7) | 0 | 0 | 0 |
| LBBB | 0 | 0 | 0 | 0 | 0 | 0 |
| RBBB | 3 (6) | 0 | 2 (13) | 0 | 0 | 1 (5) |
| QRS duration (ms) | 100 (90–110) | 95 | 100 (90–114) | 110 (100–120) | 110 (100–120) | 97.5 (88–102) |
| QRS≥120 ms | 6 (12) | 0 | 3 (20) | 2 (25) | 2 (33) | 0 |
| LVH | 2 (4) | 0 | 1 (7) | 1 (12) | 0 | 0 |
| ICD implantation | 29 (56) | – | 9 (60) | 3 (37) | 4 (66) | 13 (65) |
| Primary prevention | 20 (38) | – | 2 (13) | 3 (37) | 4 (66) | 11 (55) |
| Secondary prevention | 9 (27) | – | 7 (47) | 0 | 0 | 2 (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.