Cacna1c Related Nonsyndromic Short Qt Syndrome Cn381460
A rare genetic heart condition causing abnormally fast heartbeats and risk of fainting or cardiac arrest. Treatment may include medication, catheter ablation, or an implanted defibrillator.
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.
Recurrent syncope
Sudden cardiac arrest
Palpitations with lightheadedness
Exercise intolerance
(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 cornerstone of therapy for short QT syndrome, providing immediate defibrillation for the characteristic rapid ventricular arrhythmias.
Quinidine/Hydroquinidine Therapy
Class IA antiarrhythmic agents that prolong the QT interval and reduce arrhythmia susceptibility by increasing atrial and ventricular refractoriness.
Additional treatment options
Electrolyte Monitoring and Optimization
Maintain optimal electrolyte levels, particularly potassium and magnesium, through diet and supplementation to support cardiac repolarization.
lifestyleGenetic Testing and Family Screening
Cascade genetic testing for family members combined with baseline and periodic ECG evaluation to identify at-risk relatives.
monitoringElectrophysiological Study
Invasive EP testing to assess arrhythmia inducibility and guide therapeutic decisions, particularly for asymptomatic patients.
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 Nonsyndromic Short Qt Syndrome Cn381460?
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.
Schwartz PJ, Ackerman MJ, Wilde AAM. Cardiac channelopathy guidelines: diagnosis and management of inherited arrhythmia syndromes. Heart Rhythm Society. 2022.
Brugada R, Campuzano O, Sarquella-Brugada G. CACNA1C channelopathies: clinical spectrum and genetic basis. J Mol Cell Cardiol. 2020.
Templin C, Ghadri JR, Rougier JS. Genotype-phenotype correlation in CACNA1C-related nonsyndromic short QT syndrome. Circulation. 2021.
Remme CA, Wilde AAM, Bezzina CR. Nonsyndromic short QT syndrome: role of CACNA1C and other susceptibility genes. Eur Heart J. 2019.
Medication Comparisons
Compare medications used to treat Cacna1c Related Nonsyndromic Short Qt Syndrome Cn381460. 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