Catecholaminergic Ventricular Tachycardia (CPVT)

Introduction

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, but life-threatening inherited arrhythmia. It is triggered by physical activity or emotional stress and is not associated with structural heart disease.

Causes and Mechanism

CPVT is primarily caused by mutations in genes related to calcium signaling in heart muscle cells, such as RYR2 or CASQ2. These mutations result in abnormal calcium handling within cardiomyocytes, leading to ventricular tachycardia (VT) during periods of high catecholamine (adrenaline) activity.

Symptoms

  • Palpitations
  • Syncope (fainting), especially during exercise or emotional stress
  • Sudden cardiac arrest (in severe cases)

Diagnosis

  • Electrocardiogram (ECG): Typically normal at rest
  • Exercise stress test: Provokes arrhythmia
  • Holter monitoring: Detects episodes of VT
  • Genetic testing: Identifies relevant mutations
  • Echocardiogram & MRI: Rule out structural heart disease

Treatment

1. Beta-blockers (e.g., Nadolol, Propranolol)

  • Reduce catecholamine-induced arrhythmias

    2. Calcium channel blockers (e.g., Verapamil)

  • May provide additional benefits

    3. Flecainide

  • Used for more severe cases resistant to beta-blockers

    4. Implantable Cardioverter-Defibrillator (ICD)

  • For high-risk patients with a history of cardiac arrest

    5. Lifestyle Modifications

  • Avoid strenuous exercise and emotional stress

  • Family screening due to genetic nature

Conclusion

CPVT is a serious but manageable condition with early diagnosis and appropriate treatment. Patients should follow medical advice closely and ensure regular follow-ups.

Source recommendations

1. 2022 AHA/ACC/HRS Guideline for the Management of Patients With Ventricular Arrhythmias

  1. https://pubmed.ncbi.nlm.nih.gov/29084733/
  2. https://www.sciencedirect.com/science/article/pii/S2405500X22010945
  3. https://www.ahajournals.org/doi/abs/10.1161/CIR.0000000000000549
  4. https://www.jacc.org/doi/10.1016/j.jacc.2017.10.053
  5. https://www.escardio.org/Guidelines/Clinical-Practice-Guidelines/Ventricular-Arrhythmias-and-the-Prevention-of-Sudden-Cardiac-Death

2. 2022 ESC Guidelines for the Management of Patients with Ventricular Arrhythmias

  1. https://academic.oup.com/eurheartj/article/43/40/3997/6675633
  2. https://pubmed.ncbi.nlm.nih.gov/36017572/
  3. https://www.escardio.org/Guidelines/Clinical-Practice-Guidelines/Ventricular-Arrhythmias-and-the-Prevention-of-Sudden-Cardiac-Death
  4. https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2022/09/02/14/23/2022-ESC-Guidelines-for-VAs-ESC-2022
  5. https://www.sciencedirect.com/science/article/pii/S2405500X22010945

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