Sudden Death
It is one of the most frequent causes of death in the general population (1 case per 1000 inhabitants in Western countries each year) and is responsible for half of all cardiac deaths.
Sudden death in young people is rare, but people at risk may have hidden heart defects or abnormalities of the heart. Sudden deaths often occur during physical activity, such as a sporting event.
Learn more about the causes of sudden death.
Arrhythmogenic Cardiomyopathy
Hypertrophic Cardiomyopathy
Dilated Cardiomyopathy
Long QT syndrome
Wolff-Parkinson-Whyte Syndrome
Brugada Syndrome
The most frequent causes
The causes of sudden cardiac death in young people can be diverse. In about two-thirds of the cases, the medical examiner discovers during the autopsy that death was due to a cardiac abnormality.
For a variety of reasons, something-such as a structural heart defect-causes the heart to beat out of control. This abnormal heart rhythm is known as ventricular fibrillation. Some specific causes of sudden cardiac death in young people.
Arrhythmogenic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM)
Dilated Cardiomyopathy
Arrhythmogenic Cardiomyopathy is a heart disease that causes arrhythmias and is usually characterized by changes in the right ventricle. It is a genetic disease: five genes have been identified so far.
The genetic mutation induces the production of defective cardiac proteins that in the long run cause damage and destruction of heart cells.
Read more
A reparative process ensues in which cardiac cells are replaced by fibrous tissue and fatty tissue.
However, this process usually does not affect all cardiac cells but is sparsely distributed; as a result, areas with fibrosis and fat replacement may be surrounded by areas of cardiac cells.
In most cases the disease is transmitted in an autosomal dominant mode, which means that if either parent carries the disease each child has a 50% chance of inheriting the gene-disease mutation.
Inheriting the mutation does not automatically mean being sick, but rather being at risk of developing the disease. In many cases it happens that the disease is not expressed and cardiological tests are negative. However, it should be considered that usually the clinical signs of Arrhythmogenic Cardiomyopathy of the Right Ventricle arise in adolescence and early youth (15-25 years of age) so it is rare to find people sick at an early age and it is necessary to have periodic checkups to assess a possible onset of the disease over time.
Hypertrophic Cardiomyopathy (HCM) is a disease in which the heart muscle (myocardium) becomes enlarged, making it more difficult for the heart to pump blood.
Generally nonfatal in most people, it is the most common cause of heart-related sudden death in people under 30. It is the most common cause of sudden death in athletes but often goes unnoticed.
Dilated Cardiomyopathy is a disease characterized by dilatation of both ventricles, which have reduced systolic function (especially the left ventricle).
In the long run, islands of fibrosis appear within the myocardium, which are the trigger for arrhythmias, even major ones.
Read more
Most cases evolve, however, to heart failure, which is the leading cause of mortality in this disease.
There are primary forms (probably genetically based) and secondary forms, caused by the chronic evolution of other diseases (myocardial infarction, myocarditis) or by the use of toxic substances.
Coronary artery abnormalities: When people are born with arteries of the heart (coronary arteries) that are abnormal, the arteries can become compressed during exercise and do not provide adequate blood flow to the heart.
Arrhythmogenic Cardiomyopathy
Arrhythmogenic Cardiomyopathy is a heart disease that causes arrhythmias and is usually characterized by changes in the right ventricle. It is a genetic disease: five genes have been identified so far.
The genetic mutation induces the production of defective cardiac proteins that in the long run cause damage and destruction of heart cells.
Read more
A reparative process ensues in which cardiac cells are replaced by fibrous tissue and fatty tissue.
However, this process usually does not affect all cardiac cells but is sparsely distributed; as a result, areas with fibrosis and fat replacement may be surrounded by areas of cardiac cells.
In most cases the disease is transmitted in an autosomal dominant mode, which means that if either parent carries the disease each child has a 50% chance of inheriting the gene-disease mutation.
Inheriting the mutation does not automatically mean being sick, but rather being at risk of developing the disease. In many cases it happens that the disease is not expressed and cardiological tests are negative. However, it should be considered that usually the clinical signs of Arrhythmogenic Cardiomyopathy of the Right Ventricle arise in adolescence and early youth (15-25 years of age) so it is rare to find people sick at an early age and it is necessary to have periodic checkups to assess a possible onset of the disease over time.
Hypertrophic cardiomyopathy (HCM)
Hypertrophic Cardiomyopathy (HCM) is a disease in which the heart muscle (myocardium) becomes enlarged, making it more difficult for the heart to pump blood.
Generally nonfatal in most people, it is the most common cause of heart-related sudden death in people under 30. It is the most common cause of sudden death in athletes but often goes unnoticed.
Dilated Cardiomyopathy
Dilated Cardiomyopathy is a disease characterized by dilatation of both ventricles, which have reduced systolic function (especially the left ventricle).
In the long run, islands of fibrosis appear within the myocardium, which are the trigger for arrhythmias, even major ones.
Read more
Most cases evolve, however, to heart failure, which is the leading cause of mortality in this disease.
There are primary forms (probably genetically based) and secondary forms, caused by the chronic evolution of other diseases (myocardial infarction, myocarditis) or by the use of toxic substances.
Coronary artery abnormalities: When people are born with arteries of the heart (coronary arteries) that are abnormal, the arteries can become compressed during exercise and do not provide adequate blood flow to the heart.
Other causes of sudden death
There are other causes of sudden cardiac death in young people. These include structural abnormalities of the heart, including unrecognized congenital heart disease and heart muscle abnormalities.
Other causes include inflammation of the heart muscle, which can be caused by viruses and other diseases. In addition to Long QT syndrome, there are other abnormalities of the heart’s electrical system, such as Wolff-Parkinson-Whyte Syndrome, Brugada Syndrome, and Arrhythmogenic Cardiomyopathy, which can cause sudden death.
Long QT syndrome (LQTS)
Wolff-Parkinson-Whyte Syndrome (WPW)
Brugada Syndrome
Long QT syndrome (LQTS) is an inherited disease that results in a heart rhythm disturbance that can cause fast, chaotic heartbeats. Fast heartbeats can lead to fainting.
In some cases, your heart rhythm may be so irregular that it can cause sudden death. Young people with long QT syndrome have an increased risk of sudden death.
Wolff-Parkinson-Whyte syndrome (WPW) is a congenital disorder characterized by the presence of abnormal electrical fibers (accessory pathways) bridging atria and ventricles, which can be responsible for even life-threatening arrhythmias!
The prevalence among the population is about 0.1-0.2%. It can be asymptomatic or manifest with palpitations, at any age.
Read more
Some of those with this electrocardiographic abnormality experience episodes of tachycardia, with elevated heart rate, of sudden onset and abrupt termination.
Themost unfortunate eventuality is the onset of Sudden Cardiac Death, which can degenerate into ventricular fibrillation with an inauspicious outcome; this eventuality is considered extremely rare under the age of 12 years if there is no concomitant organic heart disease.
Sudden death is an unpredictable event based on the electrocardiogram: it can occur in 10-25% of cases as the first arrhythmic event, and in symptomatic patients, a risk of 0.0025 per year can be predicted, which means that a symptomatic young person 15 years of age has a 5% risk of sudden death in 20 years; for asymptomatic people, that risk at 20 years is 0.5-1.25%!
Brugada Syndrome is a pathology of the heart’s electrical conduction system that can cause fainting and sudden cardiac death at a young age, mainly between 20 and 40 years.
It is more common in males, and the prevalence in the typical electrocardiogram population is about 0.016%.
Read more
Only theelectrocardiographic appearance (the most common and most feared form is type 1, which has the typical “saddle” appearance on the electrocardiogram) does not realize the syndrome with danger of sudden cardiac arrest, so the attitude to take is still debated among scholars.
In 6-10% of patients who survive cardiac arrest, no structural heart disease can be demonstrated.
“Brugada syndrome” appears to be responsible for episodes of idiopathic ventricular fibrillation (VF ) in between 3 and 60 percent of cases.
The arrhythmic events of the disease they usually occur at night, a characteristic for which this condition is called by different names in various Asian regions: “Lai Tai” (death in sleep) in Thailand, “Bangungut” (screaming followed by sudden death during sleep) in the Philippines, “Pokkuri” (sudden and unexpected death during the night) in Japan.
This disease, which can manifest in various forms, also runs in families; one of the genes identified so far as responsible for the disease is located on chromosome 3 and results in an alteration in sodium transport channels at the level of the cell membrane.
Long QT syndrome (LQTS)
Long QT syndrome (LQTS) is an inherited disease that results in a heart rhythm disturbance that can cause fast, chaotic heartbeats. Fast heartbeats can lead to fainting.
In some cases, your heart rhythm may be so irregular that it can cause sudden death. Young people with long QT syndrome have an increased risk of sudden death.
Wolff-Parkinson-Whyte Syndrome (WPW)
Wolff-Parkinson-Whyte syndrome (WPW) is a congenital disorder characterized by the presence of abnormal electrical fibers (accessory pathways) bridging atria and ventricles, which can be responsible for even life-threatening arrhythmias!
The prevalence among the population is about 0.1-0.2%. It can be asymptomatic or manifest with palpitations, at any age.
Read more
Some of those with this electrocardiographic abnormality experience episodes of tachycardia, with elevated heart rate, of sudden onset and abrupt termination.
Themost unfortunate eventuality is the onset of Sudden Cardiac Death, which can degenerate into ventricular fibrillation with an inauspicious outcome; this eventuality is considered extremely rare under the age of 12 years if there is no concomitant organic heart disease.
Sudden death is an unpredictable event based on the electrocardiogram: it can occur in 10-25% of cases as the first arrhythmic event, and in symptomatic patients, a risk of 0.0025 per year can be predicted, which means that a symptomatic young person 15 years of age has a 5% risk of sudden death in 20 years; for asymptomatic people, that risk at 20 years is 0.5-1.25%!
Brugada Syndrome
Brugada Syndrome is a pathology of the heart’s electrical conduction system that can cause fainting and sudden cardiac death at a young age, mainly between 20 and 40 years.
It is more common in males, and the prevalence in the typical electrocardiogram population is about 0.016%.
Read more
Only theelectrocardiographic appearance (the most common and most feared form is type 1, which has the typical “saddle” appearance on the electrocardiogram) does not realize the syndrome with danger of sudden cardiac arrest, so the attitude to take is still debated among scholars.
In 6-10% of patients who survive cardiac arrest, no structural heart disease can be demonstrated.
“Brugada syndrome” appears to be responsible for episodes of idiopathic ventricular fibrillation (VF ) in between 3 and 60 percent of cases.
The arrhythmic events of the disease they usually occur at night, a characteristic for which this condition is called by different names in various Asian regions: “Lai Tai” (death in sleep) in Thailand, “Bangungut” (screaming followed by sudden death during sleep) in the Philippines, “Pokkuri” (sudden and unexpected death during the night) in Japan.
This disease, which can manifest in various forms, also runs in families; one of the genes identified so far as responsible for the disease is located on chromosome 3 and results in an alteration in sodium transport channels at the level of the cell membrane.
