How I cured my inherited arrhythmia syndromes? How to treat inherited arrhythmia syndromes? Let’s find out about inherited arrhythmia syndrome symptoms and treatment!
Inherited Arrhythmia Syndromes Definition
Inherited arrhythmia syndromes meaning – What are inherited arrhythmia syndromes? Inherited primary arrhythmia syndromes are electrical problems in the heart caused by changes in the structure and function of the cardiac ion channels.
Inherited Arrhythmia Syndromes Causes
What causes inherited arrhythmia syndromes? What is the most common cause of inherited arrhythmia syndromes?
Due to gene mutations in cardiac channels that cause abnormal regulation of electrolytes across the cardiac cell membrane, inherited arrhythmia syndromes can cause dangerous ventricular arrhythmias that could kill the person. Congenital long QT syndrome is a rare disease that affects 1 in 2500 live births. It is marked by a long QT interval (usually more than 470 msec) and ventricular arrhythmia, usually polymorphic ventricular tachycardia.
Long QT syndrome is usually caused by taking antiarrhythmic drugs (like sotalol or dofetilide), methadone, antidepressants, or certain antibiotics; abnormal electrolytes; myocardial ischemia; or having a very slow heart rate. Brugada syndrome is usually caused by a problem with a sodium channel gene. It is responsible for up to 20% of sudden cardiac deaths in people who do not have structural heart disease.
Arrhythmogenic RV cardiomyopathy is inherited cardiomyopathy that mostly affects the right ventricle and is marked by areas of myocardial replacement with fibrosis and fatty tissue that often cause ventricular arrhythmia. In addition, catecholaminergic polymorphic ventricular tachycardia is a rare but important exercise-related cause of sudden cardiac death.
Inherited Arrhythmia Syndromes Diagnosis
- This category includes long QT syndrome, Brugada syndrome, arrhythmogenic RV cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia.
- Genetic testing for people with congenital long QT syndrome based on family history, ECG or exercise testing, or a very long QT interval (> 500 msec) on a series of ECGs.
- Patients with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia should be given an oral beta-blocker for a long time (nadolol or propranolol). ICD is recommended for people with ventricular arrhythmia or fainting despite medical treatment.
Inherited Arrhythmia Syndromes Symptoms
Signs and symptoms of inherited arrhythmia syndromes – What are some symptoms of inherited arrhythmia syndromes?
Patients with an inherited arrhythmia syndrome may have no symptoms or have palpitations, sustained tachyarrhythmia, syncope, or sudden cardiac arrest. When young people have syncopal episodes, they may be misdiagnosed as having a disorder that causes primary seizures.
Everyone should have a full review of their personal and family history. When doing a 12-lead ECG, the ST segment, T wave, and QT interval should be carefully checked for any problems. A patient with long QT syndrome is at high risk if their corrected QT interval is longer than 500 msec on multiple ECGs and there is no other cause (like a drug or electrolyte problem).
Ambulatory ECG monitoring can be used to look for ventricular arrhythmias and see if the QT interval or T wave changes over time. When a patient is thought to have long QT syndrome, an exercise ECG may be done to see if the QT interval doesn’t shorten as it should when the heart rate goes up. When the cause of a sudden cardiac arrest is thought to be heritable, genetic testing under a multidisciplinary genetics team is recommended to find the cause and find first-degree relatives who are at risk of getting the same disease.
Inherited Arrhythmia Syndromes Treatment and Management Guidelines
Treatment of inherited arrhythmia syndromes – What is the best treatment for inherited arrhythmia syndromes?
Treatment for torsades de pointes, a type of acute polymorphic ventricular tachycardia, differs from treatment for other types of ventricular tachycardia. Antiarrhythmics of classes LA, LC, or III that lengthen the QT interval should not be used or should be stopped immediately in people with long QT syndrome.
If someone has long QT syndrome or catecholaminergic polymorphic ventricular tachycardia, beta-blockers given through an IV may be able to stop an electrical storm. Likewise, increasing the heart rate with a beta-agonist (dopamine or isoproterenol) infusion or temporary pacing of the atria or ventricles is a good way to break and stop the rhythm.
Patients with inherited arrhythmia syndromes are treated long-term based on whether or not they have high-risk symptoms.
The main treatment for people with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia is beta-blockers, especially propranolol or nadolol. If beta-blockers don’t work or don’t work well for a patient, a cervicothoracic sympathectomy might be an option. Unfortunately, there is no reliable drug treatment for Brugada syndrome. The best way to stop arrhythmias is to treat any triggers immediately, especially fever.
Antiarrhythmic drugs should not be given to people with inherited arrhythmia syndromes unless they have specific genetic problems that a specialist knows about. Patients with an inherited arrhythmia syndrome with sudden cardiac arrest as their first sign should get an ICD implanted. Despite medical treatment, an ICD should be considered for people with syncope or ventricular arrhythmias.
Any patient with known or suspected inherited arrhythmia syndrome or severely corrected QT interval prolongation (greater than 500 msec on serial ECGs) should be sent to a cardiologist or cardiac electrophysiologist.
I hope you understand inherited arrhythmia syndrome symptoms and treatment guidelines.