Table 2.
Clinical manifestations | Triggers | Paraclinics | ECG canonical pattern | First intention treatment | References | |
---|---|---|---|---|---|---|
Brugada syndrome (BrS) | Ventricular fibrillation or aborted sudden cardiac death, syncope, nocturnal agonal respiration, palpitations | Rest or sleep, febrile state, vagotonic conditions | ST-segment elevation on right precordial leads (V1 and V2) | Implantable cardioverter-defibrillator (ICD) | Brugada and Brugada, 1992; Antzelevitch et al., 2005 | |
Type 3 Long QT syndrome (LQTS3) | Polymorphic ventricular tachycardia (torsades de pointes), ventricular fibrillation, syncopes, sudden death | Rest or sleep, bradycardia, hypokaliemia, drugs prolonging QT interval | Prolonged QT interval | β-blockers (with or w/o mexiletine) | Wang et al., 1995; Amin et al., 2013; Giudicessi and Ackerman, 2013 | |
Arrhythmic Dilated Cardiomyopathy | Systolic dysfunction, left ventricular enlargement or dilatation. Multiple arrhythmias (text) | For MEPPC: rest (exercise suppresses PVCs) | For MEPPC: Quinidine Amiodarone | McNair et al., 2011; Laurent et al., 2012; Mann et al., 2012; Nair et al., 2012; Beckermann et al., 2014 |
This list is not exhaustive, but corresponds to pathologies caused by Nav1.5 mutations that are homologous to mutations in Nav1.4 (cf. Tables 4, 5).