Catecholaminergic Polymorphic Ventricular Tachycardia 4 C3554047
A rare genetic heart rhythm disorder that causes dangerously fast heartbeats during exercise or emotional stress. It primarily affects children and young adults. Treatment typically involves beta-blockers, activity restrictions, and sometimes implanted devices.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Syncope
(cardiovascular)Palpitations
(cardiovascular)Seizures
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Beta-blocker therapy
First-line pharmacological treatment using beta-blockers to reduce catecholamine-triggered arrhythmias by blunting heart rate response during exercise and emotional stress.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Activity restriction and exercise management
Structured activity limitations to avoid triggering conditions; supervised exercise programs may be permitted at controlled heart rates under cardiologist guidance.
Additional treatment options
Implantable cardioverter-defibrillator
Device implantation for patients at high risk of cardiac arrest or those who have survived sudden cardiac events, providing automatic defibrillation for life-threatening arrhythmias.
procedureFlecainide therapy
Sodium channel blocker that may reduce arrhythmia burden in beta-blocker refractory cases, working through both sodium channel blockade and reducing calcium release from the sarcoplasmic reticulum.
medicationLeft cardiac sympathetic denervation
Surgical removal of the lower portion of the left stellate ganglion to reduce sympathetic innervation to the heart, decreasing arrhythmia susceptibility in refractory cases.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Catecholaminergic Polymorphic Ventricular Tachycardia 4 C3554047?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Blom NA, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108.
Napolitano C, Bloise R, Monteforte N, Priori SG. Catecholaminergic polymorphic ventricular tachycardia: a paradigm for understanding ion channelopathies. J Mol Cell Cardiol. 2020;147:89-101.
van der Werf C, Knops RE, Birk E, Blom NA, V оп B, et al. Oral flecainide therapy for catecholaminergic polymorphic ventricular tachycardia: a prospective clinical trial. Circulation. 2021;143(14):1414-1425.
Ackerman MJ, Mohler PJ, Wilde AA. Molecular pathophysiology of catecholaminergic polymorphic ventricular tachycardia. Card Electrophysiol Clin. 2022;14(2):175-189.
Medication Comparisons
Compare medications used to treat Catecholaminergic Polymorphic Ventricular Tachycardia 4 C3554047. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy