Catecholaminergic polymorphic ventricular tachycardia

Treatment

What treatments are available for catecholaminergic polymorphic ventricular tachycardia?

There are a few treatment options available to those diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT).

Beta blockers

  • Have a proven efficacy in about 60% of individuals diagnosed with CPVT
  • Helps reduce catecholamine release by reducing heart rate and controlling the movement of calcium in the muscles
  • Recommended drugs include nadolol (1 - 2.5mg/kg/day with 2 doses per day) and propranolol (2-4 mg/kg/day with 3-4 doses per day)
  • Dosage should be based off each individual's personal exercise stress test results

Flecainide

  • Helps with arrhythmias and can be taken with beta-blockers
  • Effective in individuals with recurrent syncope or arrhythmias during exercise
  • There is a lack of controlled clinical trials, but the effectiveness of use in individuals in conjunction with beta-blockers is sufficient
  • Also recommended for individuals with an implantable cardioverter defibrillator (ICD)

Implantable cardioverter defibrillator (ICD)

  • An ICD is a device placed under the skin to monitor heart rate. If it detects an abnormal heart rhythm or arrhythmia, it will deliver an electric shock to restore a normal heartbeat.
  • Recommended in individuals who have experienced cardiac arrest, recurrent fainting episodes (syncope), ventricular tachycardia despite other treatment
  • Can be used as primary prevention of sudden death if beta-blockers are not effective
  • ICDs in younger patients require life-long device replacement and can be associated with device malfunction, infection, and psychological issues

Left cardiac sympathetic denervation (LCSD)

  • Works by preventing the release of catecholamines in the heart and raising the threshold for ventricular fibrillation
  • This does not change the way the heart muscle functions or the heart rate
  • Can be recommended for individuals along with the use of beta-blockers
  • Side effects of LCSD include palpebral ptosis, elevation of the left hemidiaphragm, and lack of sweating from the left arm and face
  • Recurrence of cardiac arrest have been reported in individuals with LCSD

Please consult with your primary care physician to determine which treatment plan would be best for you. For additional information on CPVT, please speak with a genetic counselor. To find a genetic counselor in the United States, please visit the Find a Genetic Counselor page on the National Society of Genetic Counselors website.

https://www.heart.org/en/health-topics/arrhythmia/prevention--treatment-of-arrhythmia/implantable-cardioverter-defibrillator-icd

Cho, Y. (2016). Left cardiac sympathetic denervation: An important treatment option for patients with hereditary ventricular arrhythmias. Journal of Arrhythmia, 32(5), 340-343.

Napolitano, C., Priori, S. G., Bloise, R. (2016). Catecholaminergic polymorphic ventricular tachycardia. Gene Reviews. Retrieved from https://www.ncbi.nlm.nih.gov/books/NBK1289/.

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