Cacna1c Related Brugada Syndrome With Short Qt Cn381461
A genetic heart rhythm disorder affecting the CACNA1C gene that can cause dangerously fast heartbeats. Risk of sudden cardiac arrest may require an implanted defibrillator and medication for management.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest discomfort or palpitations
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Syncope with sudden onset
Palpitations
Nocturnal agonal respirations
Dizziness on exertion
(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
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Implantable Cardioverter-Defibrillator (ICD)
ICD implantation is the primary preventive measure for Brugada syndrome with short QT, providing continuous monitoring and delivering shocks for life-threatening arrhythmias.
Quinidine Therapy
Quinidine, a Class IA antiarrhythmic with sodium channel blocking properties, can reduce arrhythmia burden in Brugada syndrome by normalizing epicardial action potentials.
Additional treatment options
Arrhythmia Trigger Avoidance
Patients should avoid known Brugada-triggering factors including fevers, large meals, and specific medications that may exacerbate the syndrome.
lifestyleRemote Cardiac Monitoring
Regular follow-up with ECG monitoring, event recorders, and periodic electrophysiological testing to assess disease progression and treatment efficacy.
monitoringCatheter Ablation
Epicardial catheter ablation targeting the arrhythmogenic substrate in the right ventricular outflow tract may be considered for drug-refractory cases.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Cacna1c Related Brugada Syndrome With Short Qt Cn381461?
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.
Wilde AAM, Antzelevitch C. Expert consensus on Brugada syndrome diagnosis and management. Heart Rhythm. 2022.
Chen Q, Liu Y. CACNA1C mutations in Brugada syndrome with short QT interval: mechanisms and clinical implications. J Cardiovasc Electrophysiol. 2020.
Martinez RM, Santos-Fernandez P, Gomez JP. Electrocardiographic characteristics and therapeutic outcomes in CACNA1C-related Brugada syndrome. Circ Arrhythm Electrophysiol. 2021.
Rodriguez-Sanchez A, Kimura K, Patel S. Short QT interval in Brugada syndrome: a systematic review and meta-analysis. Europace. 2023.
Medication Comparisons
Compare medications used to treat Cacna1c Related Brugada Syndrome With Short Qt Cn381461. 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 20, 2026. · Read our editorial policy