Myotonic dystrophy type II, also known as proximal myotonic myopathy (PROMM), is a genetic disorder that causes muscle weakness and wasting mainly in the proximal muscles. It is caused by a mutation in the CNBP gene on chromosome 3. While muscle weakness is the primary symptom of myotonic dystrophy type II, it can also affect other organs in the body, including the heart. Patients with myotonic dystrophy type II are at risk of developing recurrent ventricular tachycardia, a life-threatening heart rhythm disorder.
Recurrent ventricular tachycardia in patients with myotonic dystrophy type II is caused by abnormal electrical activity in the heart. The mutation in the CNBP gene can affect the functioning of ion channels in the heart, which control the flow of ions such as sodium, potassium, and calcium. This can lead to an imbalance in the electrical signals in the heart, resulting in the development of ventricular tachycardia.
The management of recurrent ventricular tachycardia in patients with myotonic dystrophy type II involves the use of antiarrhythmic medications and implantable cardioverter-defibrillators (ICDs). Antiarrhythmic medications such as beta-blockers and amiodarone can be used to control the heart rate and prevent ventricular tachycardia. However, these medications may have side effects and are not always effective in preventing recurrence of ventricular tachycardia.
ICDs are devices that are implanted under the skin and connected to the heart to monitor heart rhythm. If the device detects a dangerous heart rhythm such as ventricular tachycardia, it delivers a shock to restore normal heart rhythm. ICDs have been proven to be effective in preventing sudden cardiac death in patients with myotonic dystrophy type II and recurrent ventricular tachycardia.
What is myotonic dystrophy type II?
Myotonic dystrophy type II, also known as proximal myotonic myopathy (PROMM), is a genetic disorder that causes muscle weakness and wasting mainly in the proximal muscles. It is caused by a mutation in the CNBP gene on chromosome 3.
What is recurrent ventricular tachycardia?
Recurrent ventricular tachycardia is a life-threatening heart rhythm disorder that occurs when the heart beats too fast and irregularly. It can lead to dizziness, fainting, and sudden cardiac arrest.
How is recurrent ventricular tachycardia treated in patients with myotonic dystrophy type II?
Recurrent ventricular tachycardia in patients with myotonic dystrophy type II is managed using antiarrhythmic medications and implantable cardioverter-defibrillators (ICDs).
What are the side effects of antiarrhythmic medications?
Antiarrhythmic medications can have side effects such as nausea, dizziness, fatigue, and liver damage.
What is an implantable cardioverter-defibrillator (ICD)?
An implantable cardioverter-defibrillator (ICD) is a device that is implanted under the skin and connected to the heart to monitor heart rhythm. If the device detects a dangerous heart rhythm such as ventricular tachycardia, it delivers a shock to restore normal heart rhythm.
