Recurrent Ventricular Tachycardia Due to Myotonic Dystrophy Type Ii


    Myotonic dystrophy type II (DM2) is an autosomal dominant neuromuscular disorder characterized by muscle weakness, myotonia, and cardiac conduction abnormalities. Ventricular tachycardia (VT) is a potentially life-threatening arrhythmia that can occur in patients with DM2. VT is caused by an abnormal electrical circuit in the ventricles, the lower chambers of the heart. This abnormal circuit can cause the ventricles to beat too fast, which can lead to lightheadedness, dizziness, fainting, and even sudden cardiac death.


    The prevalence of VT in patients with DM2 is estimated to be between 20% and 30%. VT is more common in men than in women and is more likely to occur in patients with more severe DM2.

    Risk Factors

    The following factors are associated with an increased risk of VT in patients with DM2:

    ·       Male gender

    ·       Older age

    ·       More severe DM2

    ·       History of atrial fibrillation

    ·       Left ventricular dysfunction


    The symptoms of VT can vary depending on the severity of the arrhythmia and the underlying heart disease. Common symptoms include:

    ·       Palpitations

    ·       Lightheadedness

    ·       Dizziness

    ·       Fainting

    ·       Shortness of breath

    ·       Chest pain


    The diagnosis of VT is based on the patient’s history, physical examination, and electrocardiogram (ECG). An ECG is a test that records the electrical activity of the heart. In patients with VT, the ECG will show a rapid, irregular heart rhythm.

    Other tests that may be used to diagnose VT include:

    ·       Holter monitor: This is a portable ECG that can be worn for 24 hours or more to record the heart’s rhythm over time.

    ·       Echocardiogram: This is an ultrasound test that can be used to assess the heart’s structure and function.

    ·       Electrophysiology study: This is a test that uses electrical stimulation to map the heart’s electrical system and identify the source of VT.


    The treatment of VT in patients with DM2 depends on the severity of the arrhythmia and the patient’s symptoms. Treatment options include:

    ·       Medication: There are a number of medications that can be used to treat VT. These medications include beta-blockers, calcium channel blockers, and antiarrhythmic drugs.

    ·       Catheter ablation: This is a procedure that uses radiofrequency energy to destroy the abnormal electrical circuit that is causing the VT.

    ·       Implantable cardioverter-defibrillator (ICD): An ICD is a device that can be implanted in the chest to monitor the heart rhythm and deliver an electrical shock if VT occurs.


    The prognosis for patients with VT in DM2 is variable. The prognosis is better for patients who have no underlying heart disease and who can be treated with medication. The prognosis is worse for patients who have severe DM2 or left ventricular dysfunction and who require catheter ablation or ICD implantation.


    There is no cure for DM2, but there are a number of things that patients can do to reduce their risk of VT:

    ·       Regular medical follow-up: Patients with DM2 should see their doctor regularly to monitor their heart health.

    ·       Treatment of underlying heart disease: If patients have any underlying heart disease, such as atrial fibrillation or left ventricular dysfunction, they should be treated for these conditions.

    ·       Lifestyle modifications: Patients with DM2 should make lifestyle modifications, such as smoking cessation, weight loss, and regular exercise, to improve their overall health.


    VT is a potentially life-threatening arrhythmia that can occur in patients with DM2. Early diagnosis and treatment are important to improve the prognosis for patients with VT. Patients with DM2 should be aware of the symptoms of VT and should see their doctor immediately if they experience any of these symptoms.