What happens in the heart and what types there are
The syndrome is based on changes in genes encoding sodium channels in myocardial cells. Because of this, heterogeneity in cell recovery occurs in the right ventricle after each contraction, and against this background, a fast chaotic arrhythmia easily starts. Externally, the heart is normal: the valves and walls are not changed, the pumping function is preserved. There are types of ECG patterns, of which only the first is considered diagnostic - vaulted ST elevation in the right precordial leads. The second and third types require additional examination.
- Type 1 - ST vaulted, diagnostic
- Types 2 and 3 - saddle-shaped pattern, requires clarification
- Changes may appear and disappear on different ECGs
- High chest leads increase detectability
- The structure of the heart remains normal according to echocardiography.
Causes and provoking factors
The syndrome is inherited, most often in an autosomal dominant manner: the probability of transmission to a child is approximately half. At the same time, not all carriers develop seizures - much depends on additional conditions. Fever plays a special role: an increase in temperature sharply worsens the functioning of the altered channels, therefore, with any infection, the temperature is reduced actively and early. A number of drug groups can also provoke arrhythmia, and the diagnosis should be reported to every doctor.
- Hereditary transmission from one of the parents
- High temperature due to infections
- Some antiarrhythmic, psychotropic and analgesic drugs
- Alcohol abuse
- Eating a large meal before bed
- Night sleep and rest as a background for an attack
Symptoms
Most people with ECG changes do not experience any complaints, and the syndrome is discovered by chance during examination before surgery, during medical examination or after examination of relatives. Symptoms appear when an attack of arrhythmia starts: severe weakness, darkening of the eyes and loss of consciousness. Fainting in this syndrome is short, without preceding nausea and prolonged dizziness, often in a lying position. If the arrhythmia does not stop on its own, circulatory arrest develops.
- Sudden fainting, often at night and at rest
- Feeling of strong, uneven heartbeat
- Difficulty breathing at night, moaning, waking up in sweat
- Presyncope with fever
- Many carriers have no complaints at all
Diagnostics
The basis of the diagnosis is a 12-lead ECG, and the electrodes are often placed higher than usual, in the second and third intercostal spaces, so as not to miss the characteristic picture. Since the changes are not permanent, the ECG is repeated and daily monitoring is performed. EchoCG is needed to exclude structural heart diseases, which give similar changes. In specialized centers, a test with intravenous administration of a drug that reveals the latent form and genetic examination are used. A mandatory element is the examination of parents, brothers, sisters and children.
- ECG with high right precordial leads
- Repeated ECGs, including against the background of temperature
- 24-hour Holter ECG monitoring
- EchoCG to exclude structural pathology
- Drug provocation test in hospital
- Genetic testing and cascade family examination
Treatment and lifestyle
There is no medicine that eliminates the cause, so tactics are determined by the level of risk. For people without symptoms, observation and strict safety guidelines are usually sufficient. If there has already been an arrhythmic syncope or cardiac arrest, the doctor discusses the implantation of a cardioverter-defibrillator - a device that interrupts a dangerous arrhythmia. In some cases, antiarrhythmic therapy or catheter treatment is used. The decision is always made by a cardiologist-arrhythmologist after a full examination; independent selection of drugs is unacceptable and dangerous.
- Early reduction in temperature for any infection
- A list of prohibited drugs on the hands of the patient and doctors
- Avoiding alcohol and late, heavy dinners
- High-risk implantable defibrillator
- Teaching loved ones cardiopulmonary resuscitation skills
- Regular monitoring by a cardiologist and examination of relatives
