A young man suffered cardiac arrests with polymorphic ventricular tachycardia (PVT) and ventricular fibrillation (VF) triggered by ventricular premature contractions (PVCs). The arrhythmia was resistant to anti-arrhythmics, so after ICD implantation he underwent successful ablation of the triggering VE beat, which was pace-mapped to the left posterior hemi-fascicle. We review the evidence for t...

I on channels are protein valves that control an enormous range of biological function. Atomic-scale properties of channel proteins control macroscopic flows across otherwise insulating membranes of cells and subcellular compartments, and so the study of channels is a study in atomic biology. Ion channels are intrinsically multiscale devices that allow the amino acid side chains of a protein to...

Abstract Cardiac ion channelopathies encompass a set of inherited or acquired conditions that are due to dysfunction in channels their associated proteins, typically the presence structurally normal hearts. They with development ventricular arrhythmias and sudden cardiac death. The aim this review is provide historical perspective recent advances research channelopathies, Brugada syndrome, long...

Kv7.x (KCNQ) voltage-gated potassium channels form the cardiac and auditory I(Ks) current and the neuronal M-current. The five Kv7 subtypes have distinct assembly preferences encoded by a C-terminal cytoplasmic assembly domain, the A-domain Tail. Here, we present the high-resolution structure of the Kv7.4 A-domain Tail together with biochemical experiments that show that the domain is a self-as...

Cerveau et de L’Œil, Groupe Hospitalier Lariboisière – Fernand Widal, Paris, France ; 5 INSERM UMR_S 740, Université Paris Diderot, Sorbonne Paris Cité, Paris 6 Service de Neurologie, CHU de Limoges, Hôpital Dupuytren, Limoges, France; 7 Service de Cardiologie, CHU de Limoges, Hôpital Dupuytren, Limoges, France; 8 Unité Mixte de Recherche en Santé (UMR_S956), Université Pierre & Marie Curie (UP...

The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophre...

Brugada syndrome is an autosomal dominantly inherited channelopathy estimated to be responsible for 4e12% of all sudden deaths, particularly among middle-aged men. It is characterized by ST-segment elevation in the right precordial leads, in the absence of acute coronary syndrome. This report discuses a patient with subarachonoid hemorrhage who developed the characteristic electrocardiographic ...

Satisfactory treatment of patients with Dravet syndrome (DS) is often difficult. Some success can be achieved with bromides, but cognitive side effects and disturbed vigilance may limit their use. Here, we present the case of a successfully treated patient with DS and remarkable features in the course of his disease: additionally to DS, the patient was diagnosed with cystic fibrosis (CF), anoth...