catecholaminergic polymorphic ventricular tachycardia 4

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT): A Rare Inherited Arrhythmia Syndrome

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmia syndrome or channelopathy characterized by exercise- or emotional stress-induced bidirectional or polymorphic ventricular tachycardia in the setting of a structurally normal heart and a normal ECG. It is estimated to affect 1 in 10,000 people with reported mortality rates as high as 30–50% by the age of 20.

Key Features:

• Inherited condition: CPVT is an inherited genetic disorder that predisposes those affected to potentially life-threatening abnormal heart rhythms or arrhythmias.
• Exercise- or emotional stress-induced: The arrhythmias seen in CPVT typically occur during exercise or at times of emotional stress.
• Bidirectional or polymorphic ventricular tachycardia: The arrhythmias take the form of bidirectional or polymorphic ventricular tachycardia, which can be life-threatening if not treated promptly.

References:

[4] - This information is based on search result 4.

Common Signs and Symptoms of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare heart condition that can cause sudden rhythm disturbances, called arr

Diagnostic Tests for Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare heart condition that can be diagnosed through several tests. Here are some of the diagnostic tests used to diagnose CPVT:

• Treadmill Stress Testing: This test is used to provoke heart rhythm abnormalities in patients with suspected CPVT. It involves exercising on a treadmill while being monitored for any abnormal heart rhythms.
• Holter Monitoring: This test involves wearing a portable device that monitors your heart rhythm over a 24-hour period. It can help diagnose CPVT by detecting abnormal heart rhythms during periods of physical activity or stress.
• Implantable Loop Recorder (ILR): An ILR is a small device implanted under the skin to monitor your heart rhythm continuously for up to three years. It can be used to diagnose CPVT in patients who experience symptoms only occasionally.

These tests are typically performed by a cardiologist and can help confirm a diagnosis of CPVT. Genetic testing may also be recommended to establish or confirm diagnosis, clarify risks, or inform management (8).

References:

• [4] - Treadmill stress testing is used to provoke heart rhythm abnormalities in patients with suspected CPVT.
• [9] - Typical findings include polymorphic ventricular tachycardia (fast abnormal heart rhythm) during cardiac stress testing.

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Differential Diagnosis of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) can be challenging to diagnose, and it is essential to consider differential diagnoses to rule out other conditions that may present with similar symptoms. Here are some key points to consider:

• Long QT Syndrome (LQTS): CPVT has been often misdiagnosed as LQTS type 1 (LQT1), which phenotypically mimics CPVT [10]. However, the two conditions have distinct genetic and molecular mechanisms.
• Other Channelopathies: Other channelopathies, such as Brugada syndrome and short QT syndrome, can also present with abnormal heart rhythms and may be considered in the differential diagnosis of CPVT.
• Structural Heart Diseases: While CPVT is characterized by the absence of structural heart disease, other conditions like hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy can also cause syncope and sudden cardiac death.

Key Diagnostic Features to Distinguish CPVT from Other Conditions

To differentiate CPVT from other conditions, it is essential to consider the following diagnostic features:

• Family History: A family history of sudden cardiac death or unexplained syncope may suggest a genetic component and increase the likelihood of CPVT.
• Electrocardiographic (ECG) Findings: The resting 12-lead ECG can be useful in differentiating CPVT from other electrical diseases of the heart that can cause similar abnormal heart rhythms [6].
• Exercise Testing: Exercise testing may reveal abnormalities in heart rhythm and function, which can help differentiate CPVT from other conditions.

References

[4] by A Leenhardt · 2012 · Cited by 192 — CPVT occurs in children and adolescents and causes syncope and sudden cardiac death at a young age, in the absence of structural heart disease. [6] The resting 12-lead ECG is a useful test to differentiate CPVT from other electrical diseases of the heart that can cause similar abnormal heart rhythms. ... [10] by J Ozawa · 2018 · Cited by 30 — Catecholaminergic polymorphic ventricular tachycardia (CPVT) has been often misdiagnosed as long QT syndrome (LQTS) type 1 (LQT1), which phenotypically mimics ...