Catecholaminergic polymorphic ventricular tachycardia

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Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited cardiac disorder characterized by an abnormal heart rhythm known as ventricular tachycardia, which arises in response to physical activity or acute emotional stress. It is considered a rare genetic condition that can lead to syncope (fainting), cardiac arrest, or even sudden cardiac death in young individuals with structurally normal hearts.

Lead II rhythm ventricular tachycardia Vtach VT

Pathophysiology[edit | edit source]

CPVT is primarily caused by mutations in genes that encode proteins responsible for calcium regulation within the cardiac myocyte. The most commonly implicated genes are the RYR2, which encodes the ryanodine receptor, and CASQ2, which encodes calsequestrin. These mutations lead to an abnormal calcium release from the sarcoplasmic reticulum, causing the heart muscle cells to contract inappropriately.

Symptoms and Diagnosis[edit | edit source]

Individuals with CPVT may experience symptoms such as dizziness, palpitations, syncope, or seizures, typically triggered by physical or emotional stress. In some cases, the first manifestation of the disorder can be sudden cardiac death. Diagnosis is based on the clinical history, family history, genetic testing, and the characteristic findings on exercise stress testing or Holter monitoring, where exercise or stress can provoke the characteristic bidirectional ventricular tachycardia or ventricular fibrillation.

Treatment and Management[edit | edit source]

Management strategies for CPVT aim to prevent the occurrence of arrhythmias and include:

Prognosis[edit | edit source]

The prognosis for individuals with CPVT who are not treated is poor due to the risk of recurrent arrhythmias and sudden cardiac death. However, with appropriate diagnosis, genetic counseling, and treatment, many patients can lead a normal life.

Glossary of Terms[edit | edit source]

Resources[edit source]

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Contributors: Prab R. Tumpati, MD