Sudden cardiac death has serious consequences for the patient's relatives and for society. Each year in Europe and the USA, around 350 000 people, or around 0.1% of the general population, have an out-of-hospital cardiac arrest, of which only a small percentage survive with no sequelae.
The etiology of sudden death has been extensively studied at a population level. Ischemic heart disease occupies a prominent position and is responsible for up to 70% of these deaths; other structural heart diseases make up 10%, and primary arrhythmias cause a further 10%.
In young patients (less than 35 years), in whom the incidence of sudden death is 100 times lower than in the general population, arrhythmic etiology, mainly from channelopathies, in the absence of structural heart disease is much more common and is the predominant cause of sudden death in patients aged between 14 and 25 years. In older patients channelopathies such as Brugada syndrome are more likely to occur in the fourth decade of life.
The channelopathies responsible for sudden cardiac death are: Long QT syndrome, Short QT syndrome, Brugada syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia syndrome and Early repolarisation Syndrome.
Early identification and risk stratification is of major importance in patients with a channelopathy who remain asymptomatic, for several reasons. First, sudden death may be the first manifestation of the disease, with no previous warning symptoms. In addition, it should be taken into account that when we diagnose a patient with a hereditary disease, we also diagnose their family. Identification of an individual with this condition must be accompanied by meticulous familial screening. It is true that in these diseases the risk of sudden death decreases with age, but even a diagnosis in an elderly patient is relevant, as it allows identification of the disease in relatives and their appropriate work-up.
The scope of the theme is to collect state-of-the art papers on what we know so far for all types of Long QT syndromes, Brugada Syndrome, Early repolarisation Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia and Short QT syndrome. Furthermore, our aim is to provide further insight on the current research areas and which should be the future perspectives.
Sudden cardiac death has serious consequences for the patient's relatives and for society. Each year in Europe and the USA, around 350 000 people, or around 0.1% of the general population, have an out-of-hospital cardiac arrest, of which only a small percentage survive with no sequelae.
The etiology of sudden death has been extensively studied at a population level. Ischemic heart disease occupies a prominent position and is responsible for up to 70% of these deaths; other structural heart diseases make up 10%, and primary arrhythmias cause a further 10%.
In young patients (less than 35 years), in whom the incidence of sudden death is 100 times lower than in the general population, arrhythmic etiology, mainly from channelopathies, in the absence of structural heart disease is much more common and is the predominant cause of sudden death in patients aged between 14 and 25 years. In older patients channelopathies such as Brugada syndrome are more likely to occur in the fourth decade of life.
The channelopathies responsible for sudden cardiac death are: Long QT syndrome, Short QT syndrome, Brugada syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia syndrome and Early repolarisation Syndrome.
Early identification and risk stratification is of major importance in patients with a channelopathy who remain asymptomatic, for several reasons. First, sudden death may be the first manifestation of the disease, with no previous warning symptoms. In addition, it should be taken into account that when we diagnose a patient with a hereditary disease, we also diagnose their family. Identification of an individual with this condition must be accompanied by meticulous familial screening. It is true that in these diseases the risk of sudden death decreases with age, but even a diagnosis in an elderly patient is relevant, as it allows identification of the disease in relatives and their appropriate work-up.
The scope of the theme is to collect state-of-the art papers on what we know so far for all types of Long QT syndromes, Brugada Syndrome, Early repolarisation Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia and Short QT syndrome. Furthermore, our aim is to provide further insight on the current research areas and which should be the future perspectives.
