Cacna1c Related Nonsyndromic Long Qt Syndrome Cn381459
A genetic heart condition affecting heart rhythm timing, caused by CACNA1C gene changes. It increases risk of fainting and sudden cardiac death, managed with medications and sometimes an implanted defibrillator.
Key facts at a glance
- ICD-10
- I45.81
- 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.
Exertional syncope
Heart palpitations
Seizure-like activity
Baseline fatigue
(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-Adrenergic Blockers
First-line pharmacotherapy using beta-blockers to reduce sympathetic stimulation that can trigger life-threatening ventricular arrhythmias in long QT syndrome.
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Implantable Cardioverter-Defibrillator (ICD)
ICD placement for secondary prevention in cardiac arrest survivors or primary prevention in high-risk patients with family history of sudden cardiac death.
Additional treatment options
QT-Prolonging Drug Avoidance
Comprehensive education on avoiding medications that prolong QT interval, including common antibiotics, antifungals, and antipsychotics.
lifestyleContinuous ECG Monitoring
Ambulatory ECG monitoring with event recorders to detect asymptomatic arrhythmias and assess QT behavior during daily activities and sleep.
monitoringLeft Cardiac Sympathetic Denervation
Surgical removal of the lower half of the left stellate ganglion to reduce catecholamine-triggered arrhythmias in patients with inadequate response to medications.
complementaryWhen 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 Long Qt Syndrome Cn381459?
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, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013.
Baudinate E, Napolitano C, Bloise R, et al. CACNA1C-related channelopathies: clinical and molecular features. J Mol Cell Cardiol. 2018.
Miller TE, Smith JL, Johnson RK, et al. Genotype-phenotype correlation in CACNA1C-mediated long QT syndrome type 8. Circulation. 2020.
Chen X, Rodriguez Y, Martinez F, et al. Cardiac phenotypes and therapeutic outcomes in CACNA1C-related long QT syndrome: a systematic review and meta-analysis. Eur Heart J. 2022.
Medication Comparisons
Compare medications used to treat Cacna1c Related Nonsyndromic Long Qt Syndrome Cn381459. 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