TABLE 2.
Differential diagnosis between ARVC and Brugada syndrome
| Clinical characteristics | ARVC | Brugada syndrome |
|---|---|---|
| Age | 25–35 | 35–40 |
| Sex (male/female) | M > F (3:1) | M > F (8:1) |
| Distribution | Worldwide (Italy) | Worldwide (southeast Asia) |
| Inheritance | AD (AR) | AD |
| Chromosomes | 1, 2, 3, 10, 1417 | 3 |
| Gene | hRYR2, plakobin, desmoplakin | SCN5A |
| Symptoms | Palpitations | Syncope |
| Syncope | Cardiac arrest | |
| Cardiac arrest | ||
| Circumstances | Effort | Rest |
| Imaging | Morpho-functional RV (and LV) abnormalities | Normal |
| Pathology | Fibrofatty replacement | Normal |
| ECG repolarization | Inverted T-waves in right precordial leads | High take-off ST-segment V1–V3 |
| ECG depolarization | Epsilon-waves | RBBB/LAD |
| QRS prolongation | Late potentials | |
| Late potentials | ||
| AV conduction | Normal | 50% abnormal PR/HV intervals |
| Atrial arrhythmias | Late (secondary) | Early (primary 10–25%) |
| ECG changes | Fixed (mostly) | Variable |
| Ventricular arrhythmias | Monomorphic VT/VF | Polymorphic VT/VF |
| Mechanism of arrhythmias | Scar-related reentry | Phase 2 reentry |
| Drug effect Class I | ↓ | ↑ |
| Drug effect Class II | ↓ | ↑ |
| Drug effect Class III | ↓ | −/↑ |
| Drug effect Class IV | −/↓ | – |
| Beta-stimulation | ↑ | ↓ |
| Natural history | Sudden death | Sudden death |
| Heart failure |
Arrows denote changes in ST segment elevation (↑, increased; ↓, decreased; −/, small change, if any).
Modified from (4), with permission.