Electrical heart-disease: Brugada, Long QT, Short QT, Catecholaminergic Ventricular Tachycardia

Electrical Heart Disease: Brugada, Long and Short QT Syndromes

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What is Electrical Heart Disease?

Electrical heart disease refers to electrical disorders that occur in the absence of structural heart disease. It can be associated with unexplained sudden death, particularly in children and young adults. Palpitations, fainting, shortness of breath during exercise, and loss of consciousness are the most common symptoms. The most important ones are:

  • Brugada syndrome
  • Long QT syndrome
  • Short QT syndrome
  • Catecholaminergic ventricular tachycardia

Brugada Syndrome

Brugada syndrome is a genetic disease affecting the right ventricle of the heart. It carries a risk of sudden cardiac death, particularly at night or during sleep. It shows characteristic changes on ECG. Most patients may be asymptomatic, and diagnosis is usually made during routine check-ups or family screening. It is genetically inherited. ICD (implantable cardioverter-defibrillator) is used in treatment. Brugada syndrome occurs due to sodium channel dysfunction resulting from mutations in the SCN5A gene.

Symptoms of Brugada syndrome:

  • Syncope or near-syncope
  • Shortness of breath, particularly at night
  • Palpitations
  • Sudden cardiac arrest during sleep

Long QT Syndrome

Long QT syndrome is a genetic condition where the heart’s electrical activity lasts longer than normal. It is characterized by prolongation of the QT interval on ECG. It carries a risk of sudden cardiac arrest and can be particularly dangerous during swimming, sudden noises, or emotional stress. Some medications can further prolong the QT interval. Beta-blockers are used in treatment, and ICDs may be implanted in high-risk patients. Long QT syndrome occurs due to genetic mutations affecting ion channels in heart muscle cells.

Long QT syndrome symptoms:

  • Sudden onset of palpitation episodes
  • Fainting during exercise
  • Seizures while swimming
  • Cardiac arrest

Short QT Syndrome

Short QT syndrome is a rare genetic condition where the heart’s electrical activity is shorter than normal. It is identified by shortened QT interval on ECG. The risk of sudden cardiac death is high and can occur at any age. Antiarrhythmic medications may be used in treatment. Short QT syndrome develops due to genetic mutations affecting potassium channels.

Short QT syndrome symptoms:

  • Palpitations
  • Dizziness
  • Fainting
  • Sudden cardiac arrest

Catecholaminergic Ventricular Tachycardia

Catecholaminergic ventricular tachycardia is a dangerous arrhythmias that particularly occurs during exercise and emotional stress. It is a genetic disease and typically shows symptoms during childhood or adolescence. Fainting episodes during exercise are typical. Beta-blockers are used in treatment, and lifestyle modifications are important. ICDs may be implanted in high-risk patients. Catecholaminergic ventricular tachycardia occurs due to genetic mutations in proteins regulating calcium balance in the heart.

Catecholaminergic ventricular tachycardia symptoms:

  • Palpitations during exercise
  • Fainting triggered by emotional stress
  • Dizziness
  • Seizure-like activity
  • Sudden cardiac arrest

The common characteristic of these diseases is electrical disorders in a structurally normal heart and the risk of sudden cardiac death. People with a family history of unexplained sudden death are recommended to undergo cardiac evaluation.

Reference: Electrical Heart Disease